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question 1 (2018)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
a. Bernard-Soulier Syndrome
b. Glanzmann Thrombasthenia
c. Grey Platelet Syndrome
d. Hermansky-Pudlak Syndrome
e. MYH9-Related Disorder
f. Von Willebrand Type 1
g. Von Willebrand Type 2B
h. Von Willebrand Pseudo Type 2B
i. Von Willebrand Type 2M
i. A 3 year old adopted boy, originally from Peurto Rico, is referred to the clinic for troublesome nosebleeds. He is also seen in the gastroenterology clinic for colitis.
Hb 156, WBC 10.1, Plt 202, MPV 8 fl (normal)
Normal clotting profile.
Light Transmission Aggregometry (LTA): Absence of second wave aggregation to ADP. The ratio of ADP/ATP is reduced.
ii. A 23 year old woman is due removal of an impacted salivary gland stone under the ENT surgeons. She had significant bleeding from a laceration to her lower gums at the age of 8. She has had menorrhagia since menarche.
Hb 132, WBC 8.8, Plt 110
PT 11, APTT 33, FGN 3.8
VW Antigen 42 (normal), RiCOF 14 (low), FVIII 0.38 iu/ml (normal)
VwF Multimer analysis – Absent high and few intermediate multimers, but maintained triplet pattern.
PFA-100 >300 seconds (non-closure)
Ristocetin Induced Platelet Aggregation (RIPA):
- 0.5mg (Low dose) ristocetin, Patient platelets + cryoprecipitate = 88%
- 0.5mg (Low dose) ristocetin, Patient plasma + washed platelets = 20%
iii. A 23 year old woman is referred by the obstetricians for recurrent miscarriages. She has had recurrent bleeding episodes since childhood including needing a transfusion post-tonsillectomy.
Hb 100, WBC 7.3, Plt 257, MPV 10 fl (normal)
PT 12, APTT 32
PFA-100 >300 seconds (non-closure)
Light Transmission Aggregometry (LTA): No aggregation with ADP, Adrenaline or Collagen, but 73% aggregation with 1.5mg/ml of ristocetin.
question 2 (2018)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
j. Bernard-Soulier Syndrome
k. Chediak-Higashi Syndrome
l. GATA 1
m. Glanzmann Thrombasthenia
n. Grey Platelet Syndrome
o. Hermansky-Pudlak Syndrome
p. MYH9-Related Disorder
q. Quebec Syndrome
r. Paris-Trousseau-Jacobsen Syndrome
s. Scott Syndrome
t. Velocardiofacial Syndrome
u. Von Willebrand Type 1
v. Von Willebrand Type 2B
w. Von Willebrand Pseudo Type 2B
x. Von Willebrand Type 2M
y. Wiskott-Aldrich syndrome
i. A ten-year-old boy re-presents with recurrent episodes of petechiae and spontaneous nose bleeds. He had been seen in clinic in the past when he first started bruising. He was observed for a time before being discharged from regular follow-up.
Platelet count 80, large forms seen on film.
PFA-100 time >300 seconds
LTA shows failure to aggregate with ristocetin, but normal results with the other agonists.
ii. A 20 year old is seen in clinic and reports life-long easy bruising and bleeding. They have never required more than first aid to treat the bleeding.
Platelet count 110, MPV 15fl.
Electron microscopy shows giant, abnormal alpha granules.
iii. A two-year-old boy is admitted to paediatric intensive care with pneumonia. This is his third admission in the last six months. On examination there is widespread eczema with secondary bacterial infection.
Platelet count 50, MPV 5fl
iv. A 15 year old girl has had a mild bleeding disorder since a young age, which has recently become more problematic with menorrhagia.
Platelet count 100, MPV 15 gl with large misshapen forms seen.
Electron microscopy shows an absence of alpha granules.
v. A 20 year old man has been referred to ENT clinic for a polpectomy. He gives a history of easy bruising, frequent epistaxis and reports bleeding heavily after an appendectomy as a child.
Platelet count 160
PFA-100 closure time >300 seconds
LTA – a partial response to ristocetin only.
Flow cytometry confirms low GpIIb/IIIa density.
question 3 (2018)
For each of the following scenarios, select the translocation most likely to be associated with the diagnosis. Each answer can be used once, more than once, or not at all.
a. t(1;19)
b. t(1;22)
c. t(2;5)
d. t(3;14)
e. t(4;11)
f. t(11;14)
g. t(12;21)
h. inv(16)
i. A 52 year old man presenting with a six week history of night sweats, weight loss and small volume cervical and groin lymphadenopathy. Biopsy of a cervical lymph node shows a lymphoid infiltrate that is CD30+, CD4+, CD8+. A NPM1-ALK fusion gene is detected by FISH.
ii. A 63 year old man presents with loss of appetite and lethargy. A staging CT scan demonstrates splenomegaly and widespread small volume lymphadenopathy. His full blood count shows a lymphocytosis with Hb 105 and plt count 100. Flow cytometry of the peripheral blood identifies a lymphoid population that is CD19+, CD20+, CD5+. The subsequent lymph node biopsy stains positive for cyclin D1.
iii. A 25 year old woman admitted with fever and lethargy is found to be pancytopenic. Her blood film shows blast cells, which are CD34+, CD33+, HLA-DR+ on immunophenotyping. Bone marrow aspiration confirms the diagnosis and the morphology also reveals an eosinophilia, with several forms containing cytoplasmic crystals.
iv. A 65 year old female presents with a 4 month history of left eye proptosis and progressive visual loss. MRI shows a homogenous, well circumscribed lesion displacing adjacent orbital structures. Biopsy of the lesion demonstrates a CD20+, CD10-, CD23-, CD5- lymphoid infiltrate.
v. A 10 year old boy presents with a 2 week history of right hip pain and lethargy. His blood film shows blasts, which are TdT+, CD19+, CD10+, CD2-, CD3- by flow.
question 4 (2018)
Regarding the timing of cell free fetal DNA (cffDNA) testing for RhD negative mothers, which of the following statements is correct?
a. ccfDNA can be used to D type fetal cells from 14+2 weeks and Rh C,c,E,e and Kell from 16 weeks
b. ccfDNA can be used to D type fetal cells from 11+2 weeks and Rh C,c,E,e and Kell from 16 weeks
c. ccfDNA can be used to D type fetal cells from 11+2 weeks and Rh C,c,E,e and Kell from 14 weeks
d. ccfDNA can be used to full Rh + Kell type fetal cells from 12 weeks
e. ccfDNA can be used to full Rh + Kell type fetal cells from 14 weeks
question 5 (2018)
A 20 year old woman is being transfused red cells following a postpartum haemorrhage after the birth of her first child. During the transfusion the patient notices that the blood being transfused is Rh D positive, whereas she knows herself to be Rh D negative (as is her newborn). 100ml has been transfused. What is the correct course of action to prevent alloimmunisation?
a. Give 125IU of IM Anti-D per 1ml transfused
b. Give 100IU of IV Anti-D per 1ml transfused
c. Give 1500-2500IU of IV Anti-D and assess D+ cells by flow cytometry after 48 hours. Give further doses of anti-D as required until no detectable D+ cells remain.
d. Offer red cell exchange to patient.
e. No action is required.
question 6 (2018)
Which of the following is the correct vaccination schedule for hyposplenic patients?
a. Hepatitis B, Men ACWY, Men B, Men C/Hib, Pneumococcal, Seasonal Influenza
b. Men ACWY, Men B, Men C/Hib, Seasonal Influenza
c. Men B, Men C/Hib, Pneumococcal, Seasonal Influenza, Shingles
d. Men B, Men C/Hib, Pneumococcal, Seasonal Influenza
e. Men ACWY, Men B, Men C/Hib, Pneumococcal, Seasonal Influenza
question 7 (2018)
In the following scenarios of patients with DAT negative haemolysis, select the most likely diagnosis. Each answer can be used once, more than once, or not at all.
a. Hereditary Spherocytosis
b. Hereditary Elliptocytosis
c. Hereditary Pyropoikilocytosis
d. Overhydrated Hereditary Stomatocytosis
e. Dehydrated Hereditary Stomatocytosis
f. Cryohydrocytosis
g. Familial Pseudohyperkalaemia
h. South East Asian Ovalcytosis
i. Asymptomatic man with Hb 118g/l. Gel electrophoresis shows a defect in glycophorin 4.1.
ii. Asymptomatic woman with Hb 125g/l. Serum potassium 7.9mmol. Her father is from Thailand.
iii. A child with Hb 90g/l and moderate, chronic haemolysis that has resulted in outpatient haematology review. Gel electrophoresis shows reduced band 3 and protein 4.1. EMA binding by flow cytometry shows a patient:control ratio of 0.7.
iv. 6 month infant with Hb 70g/l. Blood film shows red cell fragmentation. EMA binding by flow cytometry shows patient:control ratio of 0.5.
v. Asymptomatic adult who reports problems with anaemia in childhood. His father is from Thailand.
question 8 (2018)
Select the most appropriate red cell product specifications for each of the following scenarios. All products can be assumed to be ABO and Rh D compatible with the recipient (and mother where applicable) and negative for any clinically significant antibodies. Each answer can be used once, more than once, or not at all.
a. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, CMV neg, Irradiated, up to 5 days old, Hct 0.7-0.85, suspended in CPD.
b. Irradiated, up to 35 days old, suspended in SAG-M.
c. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, up to 35 days old, suspended in SAG-M.
d. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, CMV neg, Irradiated, up to 35 days old, Hct 0.7-0.85, suspended in SAG-M.
e. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, Irradiated, up to 35 days old, suspended in SAG-M.
f. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, CMV neg, Irradiated, up to 5 days old, Hct 0.5-0.6, containing 100-120ml plasma, suspended in CPD.
g. Processed day 0 of collection, Known Donor, High Titre Anti-A/B negative, HbS neg, K neg, Irradiated, up to 5 days old, Hct 0.5-0.6, suspended in CPD.
i. A 10 year old boy requiring transfusion for post-operative anaemia after scoliosis surgery.
ii. A 5 day old neonate requiring red cell exchange for haemolytic disease of the fetus/newborn.
iii. An intrauterine transfusion is requiring for fetal anaemia in haemolytic disease of the fetus/newborn.
iv. A 5 year old girl requiring transfusion 15 days post allogeneic stem cell transplant for AML.
v. A 60 year old man requiring transfusion for a GI bleed. He received cladribine ten years previously for hairy cell leukaemia.
Question 9 (2018)
Which of the following gene mutations is associated with transient leukaemia of Down Syndrome (TL-DS)?
a. NPM1
b. GATA1
c. FLT3-ITD
d. RUNX1
e. CEBPA
question 10 (2018)
You are referred the blood film for a six day old neonate with a white cell count of 75 x10e9/l. The film reveals 15% immature cells which are pleomorphic with prominent nucleoli and basophilic, blebbed cytoplasm. The neonatologist tells you that the patient has a papular rash, hepatomegaly and was diagnosed prenatally with Down Syndrome.
Which of the following is the most like immunophenotype of the abnormal peripheral blood cells?
a. CD79a+, CD20+, CD5+, CD200+
b. TdT+, CD2+, CD3+, Cd7+
c. CD34+, CD33+, CD15+, HLA-DR+
d. TdT+, CD19+, CD10+, CD20+
e. CD34+, CD33+, CD36+, CD7+
question 11 (2018)
Which one of the following is the most appropriate scenario in which to recommend the use of hydroxycarbamide in sickle cell anaemia in children?
a. As a 1st line therapy for the primary prevention of stroke, regardless of TCD velocities
b. As a 1st line therapy for the primary prevention of stroke in the presence of raised TCD velocities
c. As a 1st line therapy for the secondary prevention of stroke instead of transfusion
d. As a 2nd line therapy for the secondary prevention of stroke, after an initial period of transfusion
e. As a 2nd line therapy for the primary prevention of stroke, after an initial period of transfusion
question 12 (2018)
Select the most appropriate diagnosis for each of the following cases
a. Type 1 Hereditary Haemochromatosis
b. Aceruloplasminaemia
c. Hyperferritinaemia-Cataract Syndrome
d. Type 4a Haemochromatosis (Ferroportin Disease)
e. African Iron Overload
f. Type 2 Haemochromatosis (Juvenile)
g. Neonatal haemochromatosis
h. Benign Hyperferritinaemia
i. Atransferritinaemia
j. Gaucher Disease
i. Upon retiring, a 65 year old man emigrates to the UK after spending his working life in Zimbabwe. At a first visit appointment with the GP he reports drinking 40 units/week of the local beer throughout his working life. Serum ferritin 3000 ug/l, transferrin saturations 40%, ALT 70, GGT 120, Bili 15.
ii. A 40 year old woman presents to her GP with fatigue. On examination her spleen is enlarged 7cm below the costal margin. Hb 100, platelets 130, neutrophils 1.0, ferritin 700 ug/l. Subsequent investigation finds that her white cell enzyme activity is at 15% of normal.
iii. A 40 year old man of British ethnicity is found to have a raised fasting blood sugar at a routine appointment with his GP. Subsequent tests reveal: Serum ferritin 1,500ug/l, Transferrin saturations 75%, ALT 90, Bili 30.
iv. A 20 year old man is referred to the neurologists by his GP with a history of progressive unsteadiness on his feet. Serum ferritin 1000, transferrin saturations 60%. An MRI scan of his brain shows iron deposition in the basal ganglia.
v. A 50 year old woman presents to her GP with generalised joint pains and fatigue. Serum ferritin 2,000 ug/l, transferrin saturations 40%. Subsequent investigation identifies mutation in the SCL40A1 gene.
Question 13 (2018)
When considering the use of an oral bisphosphonate as bone protection, which of the following patients starting steroids for a new diagnosis of ITP are the most suitable candidates?
Select one or more from the following:
a. A 55-year-old woman noted to have a collapse of the T12 vertebrae on an x-ray five years ago.
b. A 10-year-old girl, previously fit and well
c. A 67-year-old with a pre-imaging intermediate risk FRAX score, who is subsequently found to have normal femoral neck bone mineral density (BMD).
d. A 71-year-old man, previously fit and well
e. A 67-year-old woman, previously fit and well
question 14 (2018)
In each of the following scenarios select the most appropriate drug from the list below, assuming entry into a clinical trial is not available. Each answer may be used once, more than once or not at all.
a. Enasidenib
b. Gemtuzumab Ozogamacin
c. Nivolumab
d. Liposomal Daunorubicin + Cytarabine
e. Midostaurin
f. Pracinostat
g. Azacitidine
h. Ivosidenib
i. A previously fit and well 62 year old woman presents with a short history of lethargy and easy bruising. Her full blood count shows Hb 55g/l, WBC 180x10e9/l and Plt 32x10e9/l. A bone marrow aspirate demonstrates 95% blasts and the subsequent immunophenotype is consistent with acute myeloid leukaemia. She is started on hydroxycarbamide and five days later her WBC has fallen to 30x10e9/l. The FISH report is now available and shows a (del)5q. The patient would like to consider any alternatives to the current standard of care.
ii. A 69 year man is referred by his GP with a 9 month history of progressive lethargy and more recently he has been suffering with recurrent minor epistaxis. A recent full blood count has shown Hb 75g/l, WBC 1.2x10e9/l, Plt 40x10e9/l. A bone marrow aspirate shows hypocellular particles with dysplastic erythroid precursors and 26% blasts. The blasts express a myeloid phenotype on flow cytometry. Following discussion of available treatment options, the patient expresses a wish not to undergo intensive inpatient therapy but would like to consider any alternative treatment which might control his leukaemia.
iii. A 55 year old woman has started daunorubicin plus cytarabine (DA) chemotherapy for acute myeloid leukaemia. It is now day seven of treatment and her molecular cytogenetics have identified a FLT3-ITD mutation. She asks if any additional treatment is available to her.
iv. A 26 year old man is diagnosed with acute myeloid leukaemia. Flow cytometry of the peripheral blood shows the following blast cell phenotype: CD34+, HLA-DR+, CD133+, CD33+, CD3-, CD19-. His karyotype identifies inv(16). After initial control of his WBC with hydroxycarbamide he is ready to start intensive induction therapy and the patient asks if any other treatment is available in addition to DA chemotherapy.
Question 15 (2018, updated 2021)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
a. Adult T-Cell Leukaemia/Lymphoma (ATLL)
b. Angioimmunoblastic T-Cell Lymphoma (AITL)
c. ALK negative Anaplastic Large Cell Lymphoma
d. ALK positive Anaplastic Large Cell Lymhpoma
e. Enteropathy-associated T-Cell Lymphoma (EATL)
f. Extranodal NK/T-Cell lymphoma (ENKTL)
g. Hepatospenlic T-Cell Lymphoma
h. T/NK-Large Granular Lymphocytic Leukaemia (LGLL)
i. Peripheral T-Cell Lymphoma, Not otherwise specified (PTCL, NOS)
j. T-Cell Acute Lymphoblastic Leukaemia (T-ALL)
k. T-Cell Prolymphocytic Leukaemia (T-PLL)
i. A 65 year old man is admitted with epistaxis. He gives a short history of night sweats and rapid weight loss. Hb 100g/l, plt 50x10e9/l, WBC 5.0x10e9/L. CT scan of the trunk reveals small volume para-aortic lymphadenopathy and bilateral adrenal masses. Adrenal biopsy demonstrates a monomorphic lymphoid infiltrate, positive for CD2, cytoplasmic CD3, CD56 and EBER. Negative for surface CD4, CD8 and surface CD3. A subsequent bone marrow biopsy confirms marrow involvement.
ii. A 28 year old man gives a two week history of right hip pain, night sweats and weight loss. Hb 130g/l, plt 57x10e9/l, WBC 90x10e9/L. CT imaging shows a 9cm anterior mediastinal lymph node mass and hepatosplenomegaly. Flow cytometry of the peripheral blood identifies an abnormal lymphoid population which are positive for cytoplasmic CD3, CD5, CD7, CD9 and weakly positive for TdT. Negative for surface CD3, CD4, CD8, CD34 and HLA-DR.
iii. A 69 year old woman presents with a six week history of itch, lethargy, night sweats and progressive neck and axillary lymphadenopathy. Examination reveals hepatosplenomegaly. Hb 100g/l, plt 170x10e9/l, WBC 5.0x10e9/L. A subsequent axillary lymph node biopsy demonstrates a paracortical polymorphic infiltrate of small to medium sized lymphocytes. These cells are positive for CD2, CD3, CD4, CD5, CD10, CXCL13 and PD-1.
iv. A 50 year old woman is referred to clinic with night sweats and a rapidly enlarging lymph node in her right groin. Her full blood count is normal except for a mild lymphopenia. PET-CT reveals FDG uptake in the right groin and para-aortic lymph nodes. A lymph node biopsy shows a diffuse infiltrate of medium-large sized lymphoid cells with frequent mitotic and apoptotic bodies. The cells are positive for CD2, CD3, CD4 and strongly positive for CD30. They are negative for CD8, CD56, ALK, CD10 and PD1.
v. A 67 year old man with a past history of rheumatoid arthritis has an incidental finding of neutropenia. He is not currently on any medication for this rheumatoid. Examination reveals 5cm splenomegaly below the costal margin. Hb 130g/l, plt 205x10e9/l, WBC 3.0x10e9/L, neutrophils 0.5x10e9/L. He remains neutropenic over the following six months and requires treatment for two infections in that time. A peripheral blood film shows large lymphoid cells containing coarse azurophilic granules. Bone marrow biopsy demonstrates an interstitial lymphoid infiltrate accounting for 30% of cells seen on the trephine. These cells are positive for CD3, CD8, CD57 and KIR. Negative for CD5, CD7.
Question 16 (2018)
For each of the following scenarios, select the most likely causative antibody from the list below. Each answer may be used once, more than once or not at all.
a. IgG Anti-c
b. IgG Anti-e
c. IgG Anti-E
d. IgM High titre Anti-I
e. IgM Low titre Anti-I
f. IgG Anti-P
i. A 42 year old man presents with pre-syncopal symptoms on standing, which have occurred following three weeks of increasing lethargy. He is pale with jaundiced sclera and a palpable spleen tip. He has no past medical history and is not on any medication. Hb 55g/l, plt 210x10e9/l, WBC 6.5x10e9/l, Bilirubin 68, LDH 500. Examination of the blood film reveals marked polychromasia and spehrocytosis. The direct antiglobulin test (DAT) is IgG 4+ positive and negative for C3d. His red cell Rh phenotype is R1R1.
ii. A 60 year old woman attends her GP for a well woman check. The blood film is passed to you for medical review. There is marked cell agglutination, with a normal platelet count and unremarkable white cell appearances. The DAT is negative. The GP is surprised when you phone for further information, reporting that the patient is entirely well with no complaints.
iii. A 20 year old man who had recently recovered from influenza re-presents with a new fever, acute back pain and lethargy. Hb 67g/l, plt 310x10e9/l, WBC 5.4x10e9/l. His urine dipstick is positive for blood but subsequently microscopy is reported as normal. The DAT is 3+ positive for C3d. Further serological tests identify a biphasic antibody. The patient recovers fully after two weeks of supportive care.
iv. A 65 year old woman is referred by her GP. For the third winter running she has suffered with painful fingers when outside despite wearing gloves. Her fingers become discoloured during these episodes. She feels more fatigued during these winter months. Hb 105g/l, plt 190x10e9/l, WBC 4.2x10e9/l. Blood film shows red cell agglutination and the DAT is 3+ positive for C3d.
Question 17 (2018)
Which of the following medications has been most commonly associated with drug-induced immune haemolytic anaemia?
a. Aspirin
b. Omperazole
c. Fluconazole
d. Bisoprolol
e. Ceftriaxone
question 18 (2018)
Which of the following genetic features has the poorest median survival for patients diagnosed with myelofibrosis?
a. JAK2 V617F mutation
b. CALR mutation
c. MPL W515 mutation
d. ‘Triple Negative’ status
question 19 (2019)
A 70yo man presents with progressive lethargy and report purpuric lumps on his chest that appeared in the last 2-3 weeks. Hb 100, plt 70, WBC 0.9. Bone marrow aspirate demonstrates medium sized blasts with eccentric nuclei and 'tail-shaped' cytoplasm containing vacuoles. Flow cytometry shows the following immunophenotype: Positive for CD4, CD56, CD123 and CD303. Negative for MPO, CD13 and CD33.
What is the likely dx?
a. T Prolymphocytic Leukaemia
b. B Prolymphocytic Leukaemia
c. T Acute Lymphoblastic Leukaemia
d. Blastic Plasmacytoid Dendritic Cell Neoplasm
e. Acute Myeloid Leukaemia
f. Acute MyeloMonoblastic Leukaemia
Question 20 (2019)
Which of the following mechanisms correctly accounts for the reduced ability of low molecular weight heparin to bind to thrombin compared to unfractionated heparin?
(HMW = High Molecular Weight)
a. HMW heparin chains have a longer half-life, allowing sufficient time for thrombin binding
b. HMW heparin chains bind readily to fibrin-bound thrombin
c. Only HMW heparin chains carry the antithrombin-specific binding site
d. HMW heparin chains carry a stronger positive charge, allowing binding to thrombin.
e. Heparin chains must be of sufficient length to bridge between antithrombin and thrombin.
question 21 (2019)
All of the following patients have received an allogeneic stem cell transplant. In each case select the most appropriate blood component for transfusion. Each answer may be used once, more than once or not at all.
a. Group A high titre negative platelets, Rh D negative
b. Group A high titre negative platelets, Rh D positive
c. Group A red cells, Rh D negative
d. Group AB platelets, Rh D negative
e. Group AB platelets, Rh D positive
f. Group B high titre negative platelets, Rh D negative
g. Group B red cells, Rh D positive
h. Group O platelets, Rh D negative
i. A 30-year-old man is day +10 and his haemoglobin has fallen to 70g/l. He is blood group AB, Rh D negative. His donor is group A, Rh D negative.
ii. A 45-year-old woman is day +7 and experiencing troublesome epistaxis. Her platelet count is 15 x10e9/l. She is blood group AB, Rh D negative. His donor is group A, Rh D positive.
iii. A 65-year-old woman is Day +600 and has had an elective hip replacement. She is symptomatic from a post-operative haemoglobin of 69g/l. Her original blood group was O, Rh D negative. The donor is Group B, Rh D positive. Her current serology demonstrates that she is Group B, Rh D positive. The reverse grouping does not detect Anti-B, there is no mixed field reaction and the Direct Antiglobulin Test is negative.
iv. A 40-year-old man is unwell with fever and rigors at day +15. His platelet count is 9 x10e9/l. He is blood group B, Rh D negative. The donor is group AB, Rh D negative.
question 22 (2019)
A woman who is 16 weeks pregnant is seen in the joint haematology-obstetric clinic. She is a known carrier of haemophilia A with a historical FVIII level of 0.48 iu/ml. Select the statement that most accurately describes the normal haemostatic changes in pregnancy.
(VWF = Von Willebrand Factor)
(FGN = Fibrinogen)
a. FGN, FVIII and FIX levels increase. FX & Protein C remain stable. VWF levels decrease.
b. FGN, VIII and VWF levels increase. FIX and Protein C remain stable. Protein S levels decrease.
c. FGN, VIII and VWF levels increase. FIX levels remain stable. FX, Protein C & S levels decrease.
d. FVIII and VWF levels increase. FX & Protein C remain stable. FIX levels decrease.
e. FGN, VIII and IX levels increase. Protein C & S remain stable. VWF levels decrease.
question 23 (2019)
From the options below, select the statement that most accurately describes the recommended usage of pre-thawed fresh frozen plasma (FFP) in major trauma.
a. Once thawed, store at 2-4oC for up to 120 hours. Complete transfusion within 4 hours of removal from temperature-controlled environment. The unit may be accepted back into temperature-controlled storage on one occasion following less than 30 minutes spent outside of storage. This unit may then be re-issued provided the transfusion will complete within 4 hours of the original issue time.
b. Once thawed, store at 2-4oC for up to 160 hours. Complete transfusion within 4 hours of removal from temperature-controlled environment. The unit may be accepted back into temperature-controlled storage on one occasion following less than 60 minutes spent outside of storage. This unit may then be re-issued provided the transfusion will complete within 4 hours of the original issue time.
c. Once thawed, store at room temperature for up to 24 hours. Complete transfusion within 4 hours of issue. The unit may be accepted back into storage on one occasion following less than 30 minutes spent outside of storage. This unit may then be re-issued provided the transfusion will complete within 4 hours of the original issue time.
d. Once thawed, store at room temperature for up to 120 hours. Complete transfusion within 4 hours of issue. The unit may be accepted back into storage on one occasion following less than 30 minutes spent outside of storage. This unit may then be re-issued provided the transfusion will complete within 4 hours of the original issue time.
e. Once thawed, store at 2-4oC for up to 100 hours. Complete transfusion within 4 hours of removal from temperature-controlled environment. The unit may be accepted back into temperature-controlled storage on one occasion following less than 30 minutes spent outside of storage. This unit may then be re-issued provided the transfusion will complete within 60 minutes of the original issue time.
question 24 (2019)
Which one of the following recipient/donor pairings carries the greatest risk of CMV-related complications occurring post haematopoietic stem cell transplant?
a. CMV IgG-negative recipient of a CMV IgG-negative donor
b. CMV IgG-negative recipient of a CMV IgG-positive donor
c. CMV IgG-positive recipient of a CMV IgG-negative donor
d. CMV IgG-positive recipient of a CMV IgG-positive donor
e. CMV serological status does not affect post-transplant CMV complication rates
Question 25 (2019)
Which one of the following statements is correct regarding the risk of CMV reactivation post haematopoietic stem cell transplant?
a. The risk of CMV reactivation is higher in HLA-matched transplants, compared to HLA-mismatched
b. The risk of CMV reactivation is higher in related donor transplants, compared to unrelated donors
c. The use of alemtuzumab conditioning is associated with an increased risk of CMV reactivation
d. The risk of CMV reactivation is highest where both recipient and donor are CMV IgG-negative pre-transplant
e. A CMV IgG-negative recipient of a CMV IgG-positive donor has an 80% risk of CMV infection
Question 26 (2019)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
a. Anorexia nervosa
b. Aplastic Anaemia
c. Congenital Amegakaryocytic Thrombocytopenia
d. Diamond-Blackfan Anaemia
e. Dyskeratosis Congenita
f. Emberger Syndrome
g. Fanconi Anaemia
h. Hypoplastic Myelodysplasia
i. MonoMAC Syndrome
j. Schwachman-Diamond Syndrome
k. Thrombocytopenia with Absent Radii (TAR)
i. A seven-year-old boy is referred to clinic with lethargy and recurrent urinary tract infections. On examination he is noted to have café-au-lait spots on his back. His full blood count shows haemoglobin 100g/l, platelets 130 x10e9/l and neutrophils 0.9 x10e9/l. Subsequent chromosomal breakage analysis identified DNA cross-link hypersensitivity to diepoxybutane.
ii.Following an uncomplicated vaginal delivery at term, a new born girl suffers a seizure. She is noted to have a marked petechial rash and cranial ultrasound confirms the presence of an intracranial haemorrhage. Her platelet count is 70 x10e9/l. Over the next six months she develops worsening pancytopenia and a MPL gene mutation is identified.
iii. A fourteen-year-old girl is seen in clinic after a full blood count performed for lethargy and recurrent infections revealed haemoglobin 95 g/l, platelets 90 x10e9/l and neutrophils 0.5 x10e9/l. On examination she has discoloured, broken nails, oral leukoplakia and a fine lacy rash on chest. Her mother tells you these have been present for many years, although the girl has only recently felt unwell. You request further tests to confirm your suspected diagnosis.
iv. A twenty-year-old man presents with lethargy and recurrent episodes of epistaxis. Full blood count reveals haemoglobin 100g/l, platelets 19 x10e9/l, neutrophils 0.4 x10e9/l and reticulocytopenia. Bone marrow biopsy demonstrates a hypocellular marrow with marked dyserythropoiesis. Flow cytometry identifies a 2% red cell clone lacking CD55/CD59 surface markers.
v. A twenty-year-old is seen in A&E following a vasovagal collapse whilst out walking. Blood tests performed routinely in the emergency department find a potassium of 2.7 mmol/l, haemoglobin of 100 g/l, platelets 100 x10e9/l, neutrophils 1.4 x10e9/l. A bone marrow aspirate is haemodilute and non-diagnostic. Trephine histology shows an hypocellular marrow with fat cell atrophy and extracellular gelatinous deposits.
Question 27 (2019)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
a. Anorexia nervosa
b. Aplastic Anaemia
c. Congenital Amegakaryocytic Thrombocytopenia
d. Diamond-Blackfan Anaemia
e. Dyskeratosis Congenita
f. Emberger Syndrome
g. Fanconi Anaemia
h. Hypoplastic Myelodysplasia
i. MonoMAC Syndrome
j. Schwachman-Diamond Syndrome
k. Thrombocytopenia-Absent Radius (TAR)
i. A ten-month-old boy is referred for failure to thrive. His parents report his growth slowed during weaning, coinciding with a change in bowel habit to fatty, foul smelling stools. He has required treatment for pneumonia on two occasions since his birth. A full blood count shows panycytopenia with reticulocytopenia.
ii. A 22-year-old man presents with fever, lethargy and anorexia for four weeks, preceded by several months of productive cough. Five years previously he had been treated for a non-tuberculous mycobacterium skin infection. His full blood count shows haemoglobin 110 g/l, platelets 130 x10e9/l, neutrophils 2.0 x10e9/l, lymphocytes 0.8 x10e9/l, monocytes 0.1 x10e9/l. Subsequent genetic investigation detects a GATA2 mutation.
iii. A 51-year-old woman presents with two months of lethargy and easy bruising. Her full blood count showed pancytopenia, a film reports 7% blasts in the peripheral blood. A bone marrow aspirate is haemodilute and non-diagnostic. Trephine histology demonstrates 15% marrow cellularity with trilineage dysplastic changes. Monosomy 7 is detected on karyotyping.
iv. A six-year-old boy presents to his GP with right leg swelling. He had been seen at the practice several times in the preceding year for treatment of erysipelas. He is referred to the paediatric clinic where a full blood count shows haemoglobin 120 g/l, platelets 150 x10e9/l, neutrophils 0.4 x10e9/l and lymphocytes 0.4, x10e9/l. He is later found to have a GATA2 mutation.
v. A two-month-old girl is followed up in the paediatric clinic. She was reported to have been pale at birth, requiring two red cell transfusions in her first month of life. On examination, she was pale without jaundice. A full blood count shows a haemoglobin 26 g/l and reticulocytopenia. Bone marrow aspiration demonstrated a normocellular marrow with markedly reduced erythroid precursors. A RSP19 gene mutation was later identified.
Question 28a (2019)
Mr Stoker started warfarin one year ago following an unprovoked pulmonary embolism. He has attended the anticoagulation clinic for review of his INR control over the last twelve months. Which of the following set of results would constitute poor INR control?
a. Time in therapeutic range (TTR) of 70%
b. One INR reading of 7.5 in the last 6 months
c. One INR reading of 1.3 in the last 6 months
d. Two INR readings, 4.6 and 4.8, in the last 6 months
e. Two INR readings, 1.3 and 1.4, in the last 6 months
Question 28b (2019)
As it transpires, Mr Stoker’s INR control has been good and he continues on warfarin. Three months’ later he attends A&E with spontaneous epistaxis. Which of the following drugs is Mr Stoker most likely to have started taking since his last review in the anticoagulation clinic?
a. Sertraline
b. St John’s Wort
c. Rifampicin
d. Carbamazepine
e. Phenytoin
Question 29 (2019)
An 85-year-old male inpatient complains of new right calf pain. He is found to have a platelet count of 29 x10e9/l, having been 240 x10e9/l on admission. Eight days earlier he was admitted to hospital with acute ischaemia of his left foot. He has been managed conservatively with an intravenous heparin infusion, followed by subcutaneous dalteparin. He did not require surgical intervention, but he was started on intravenous piperacillin-tazobactam three days ago for a suspected hospital acquired pneumonia.
From the list below, choose your preferred initial management at this point. Select one answer.
a. Continue dalteparin and arrange urgent ultrasound Doppler of right leg
b. Continue dalteparin and test patient for presence of Heparin-Platelet Factor 4 antibodies
c. Stop anticoagulation, transfuse 1 units of platelets and arrange urgent ultrasound Doppler of right leg
d. Switch dalteparin to fondaparinux and test patient for presence of Heparin-Platelet Factor 4 antibodies
e. Switch dalteparin to warfarin and test patient for presence of Heparin-Platelet Factor 4 antibodies
Question 30 (2019)
For the following pregnant women with a diagnosis of antiphospholipid syndrome, select the most appropriate antithrombotic management in each case. Each answer may be use once, more than once or not at all.
(LMWH – Low Molecular Weight Heparin)
(APS - Antiphospholipid Syndrome)
a. No anticoagulant / antiplatelet therapy required
b. Prophylactic dose LMWH for seven days postpartum.
c. Aspirin throughout pregnancy
d. Aspirin + prophylactic dose LMWH from positive pregnancy test until at least 7 days postpartum
e. Aspirin + treatment dose LMWH from positive pregnancy test until at least 7 days postpartum
f. Prophylactic dose LMWH throughout pregnancy and for six weeks postpartum
g. Treatment dose LMWH throughout pregnancy and for six weeks postpartum
i. A 30-year-old woman diagnosed with APS following the spontaneous loss of three prior pregnancies.
ii. A 27-year-old woman currently taking warfarin following an unprovoked pulmonary embolus that resulted in a diagnosis of APS two years earlier.
iii. A 31-year-old woman pregnant with her second child. Her first child was delivered at 33 weeks’ gestation due to pre-eclampsia, and she was subsequently diagnosed with APS.
iv. A 36-year-old woman with a previous incidental finding of persistent antiphospholipid antibodies is now pregnant for the first time. She otherwise scores 1 point when risk assessed for antenatal thromboprophylaxis.
Question 31 (2019)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all.
a. Acute myeloid leukaemia with eosinophilia
b. Ascariasis
c. Chronic eosinophilic leukaemia with ETV6-ABL1 rearrangement
d. Chronic eosinophilic leukaemia with ETV6-FLT3 rearrangement
e. Coccidiodomycosis
f. Filariasis
g. Gleich’s Syndrome
h. Loeffler Syndrome
i. Systemic Mastocytosis
j. Wells Syndrome
i. CEL with ETV6-ABL1. A 55-year-old woman attends the haematology clinic. She has been referred by her GP with a two-month history of increasing lethargy and easy bruising. Haemoglobin 90 g/l, Platelets 100 x10e9/l, White Cell Count 15 x10e9/l, Eosinophils 2.4 x10e9/l. A bone marrow aspirate reveals 15% blasts. Eosinophils predominant amongst the mature myeloid cells. Following molecular testing the patient is started on sunitinib.
ii. A 24-year-old man is admitted to the emergency department with breathlessness and productive cough. Haemoglobin 120 g/l, Platelets 300 x10e9/l, White Cell Count 7.0 x10e9/l, Eosinophils 2.0 x10e9/l. A chest x-ray demonstrates left lower lobe pneumonia. Three weeks prior to admission the patient had been treated for strongyloides infection. He made a full and spontaneous recovery after one week.
iii. A 27-year-old woman presents to the out of hours GP service with spontaneous nose bleeds. She had been unwell for the preceding fortnight with lethargy and low-grade fever. Haemoglobin 75 g/l, Platelets 50 x10e9/l, White Cell Count 23 x10e9/l, Eosinophils 2.0 x10e9/l. A bone marrow aspirate demonstrates 35% myeloid blasts. Cytogenetics detects inversion(16).
iv. A 7-year-old refugee from Sierra Leone is seen in the paediatric clinic with worsening lymphodema of his right lower leg, present since birth. On examination, the overlying skin is indurated and hot to touch. A blood film revealed multiple sheathed worms. Treatment is initiated with a combination of ivermectin and albendazole.
v. A 37-year-old man presents to his GP with a three-month history of a recurrent itchy rash affecting his trunk and lower legs. On examination there are discrete slate-blue plaques on his lower legs and patches of urticaria. Haemoglobin 125 g/l, Platelets 320 x10e9/l, White Cell Count 9.0 x10e9/l, Eosinophils 2.3 x10e9/l. Skin biopsy demonstrated eosinophilic infiltration of the dermis with granulomatous changes and flame-shaped areas in the middermis.
Question 32 (2019)
A 52-year-old man presents with a short history of lethargy, night sweats and abdominal discomfort. On examination, he appears unwell and his abdomen is mildly distended with a central fullness on palpation. Cervical and axillary lymphadenopathy is present. A lymph node biopsy demonstrates complete effacement of the normal tissue architecture, which has been replaced by medium-sized, monomorphic cell with round nuclei, prominent nucleoli and basophilic cytoplasm containing prominent cytoplasmic vacuoles. The immunophenotype of his cells is as follows: CD19+, CD20+, CD38+, CD5-, BCL2-, TdT-.
Which of the following cytogenetic abnormalities is most likely to be present in this case?
a. 5(15;17)
b. t(8;22)
c. t(14;18)
d. t(11;14)
e. t(8;14)
Question 33 (2019)
A 50-year-old woman is referred to the haematology clinic for investigation of thrombocytopenia. She gives a five-year history of fatigue and long-standing joint pain. More recently she has developed persistent pain in her hips and booked a GP appointment after suffering three nose bleeds. On examination, the only finding is an enlarged spleen palpable 4cm below the costal margin. Investigations to date have shown:
Haemoglobin 110g/l, Platelets 68 x10e9/l, White Cell Count 3.4 x10e9/l
PT 12 sec, APTT 38 sec, Fibrinogen 0.9 g/l, Factor XI 0.48 iu/ml
IgG paraprotein 14g/l
Ferritin 550 ng/ml
X-Ray – abnormal expansion of the distal femurs bilaterally
Which of the following investigations is most likely to confirm the diagnosis?
a. Soluble IL-2 receptor levels (sCD25)
b. White cell enzyme screen
c. Bone marrow biopsy
d. Abdominal ultrasound
e. ADAMTS13 Level
Question 34 (2019)
A 28-year-old pregnant woman attends for her booking appointment. She has been feeling more tired recently and experiencing occasional headaches. She has no past medical history and her only medication is folic acid 400 micrograms daily. Her full blood count shows haemoglobin 100g/l, mean cell volume 82fl, Platelets 290 x10e9/l, White cell count 4.0 x10e9/l.
From the list below, select the most appropriate initial management for this patient.
a. Start ferrous fumerate 210mg once daily for 2-3 weeks and then re-check full blood count
b. Start ferrous fumerate 210mg twice daily for 2-3 weeks and then re-check full blood count
c. Test serum ferritin and Start ferrous fumerate 210mg once daily for 2-3 weeks if ferritin <30ug/l
d. Test serum ferritin and Start ferrous fumerate 210mg twice daily for 2-3 weeks if ferritin <30ug/l
e. Test serum ferritin and arrange for intravenous iron infusion if ferritin <30ug/l
Question 35 (2019)
What is the approximate risk of a hospitalised, RhD negative patient forming an anti-D alloantibody following exposure to RhD positive red cell transfusion?
a. <1%
b. 0-10%
c. 10-20%
d. 20-30%
e. >50%
Question 36 (2019)
Which of the following patient groups is at greatest risk of forming non-ABO red cell alloantibodies following a blood transfusion?
a. Children under the age of 1 year
b. Hospitalised, non-oncology patients
c. Patients undergoing solid organ transplantation
d. Patients with myelodysplasia
e. Patients transfused in the presence of a haematinic deficiency
Question 37 (2019)
A 35-year old man presents to the emergency department covered in spontaneous bruising and reporting rectal bleeding. He had recently started anticoagulation for a DVT. His APTT was grossly prolonged and subsequent testing revealed a Factor IX level of <0.01 iu/ml. He denies any history of bleeding prior this current episode.
What anticoagulant is the patient taking?
a. Warfarin
b. Dalteparin
c. Rivaroxaban
d. Dabigatran
e. Danaparoid
Question 38 (2020)
In each of the following cases, select one drug from the list that the patient is most likely to have received recently. Each drug may be selected once, more than once or not at all.
Normal Ranges:
Thrombin Time (TT) 12.5-14 seconds
Prothrombin Time (PT) 11-13.5 seconds
Activated Partial Thromboplastin Time (APTT) 28-36.5 seconds
Platelet count 150-370 x10e9/l
a. Abciximab
b. Dabigatran
c. Dalteparin
d. Rivaroxaban
e. Warfarin
i. TT 100 seconds, PT 15 seconds, APTT 39 seconds, platelets 170 x10e9/l
ii. TT 13 seconds, PT 25 seconds, APTT 31 seconds, platelets 350 x10e9/l
iii. TT 13.5 seconds, PT 13 seconds, APTT 43 seconds, platelets 220 x10e9/l
iv. TT 13 seconds, PT 16 seconds, APTT 38 seconds, platelets 175 x10e9/l
v. TT 12.5 seconds, PT 11.5 seconds, APTT 35 seconds, platelets 25 x10e9/l
Question 39 (2020)
For each of the following patients who present with a symptomatic, unprovoked venous thromboembolism (VTE) for the first time, state whether the statement is true or false.
(PE = Pulmonary embolism, DVT = Deep Vein Thrombosis)
a. After stopping anticoagulation, the rate of recurrent VTE is approximately 10% at one year.
b. The rate of recurrence is greater for women than for men.
c. Fatality rate when recurrent VTE occurs is approximately 15%.
d. A recurrent VTE is more likely to be a PE if the first event was a PE, as opposed to a DVT.
e. The site of DVT (proximal versus distal) does not affect the risk of recurrence.
Question 40 (2020)
For each of the following pregnant women select the most appropriate management for reducing their risk of venous thromboembolism. Each answer may be used once, more than once or not at all.
(LMWH – Low Molecular Weight Heparin)
a. No chemical thromboprophylaxis indicated
b. Prophylactic dose LMWH for 10 days post-delivery
c. Prophylactic dose LMWH for 6 weeks post-delivery
d. Prophylactic dose LMWH from 28 weeks gestation until 6 weeks post-delivery
e. Treatment dose LMWH from 28 weeks gestation until 6 weeks post-delivery
f. Prophylactic dose LMWH from start of pregnancy until 6 weeks post-delivery
g. Intermediate dose LMWH (e.g. 50-75% of treatment dose) from start of pregnancy until 6 weeks post-delivery
h. Treatment dose LMWH from start of pregnancy until 6 weeks post-delivery
i. A 29 year old woman with a history of deep vein thrombosis (DVT) occurring five years ago and associated at the time with use of the combined oral contraceptive pill. She received three months anticoagulation. Recently, her sister also developed a DVT. Subsequent tests identified that both individuals have low antithrombin levels and share the same SERPINC1 mutation.
ii. A 25 year old woman pregnant with her first child, usually fit and well with no personal or family history of thrombosis. She is a non-smoker and her body mass index (BMI) is 22. Her pregnancy proceeds without complication. Her baby is delivered by emergency caesarean section after a prolonged labour of greater than 24 hours.
iii. A 36 year old woman, pregnant with her fourth child. She is a current smoker and her BMI is 41. Her past medical history includes symptomatic varicose veins. She has no personal or family history of thrombosis.
iv. A 34 year old woman, pregnant with her first child. She is a non-smoker with no past medical history and no family history of thrombosis. Her BMI is 25.
v. A 29 year old woman, pregnant with her second child. She is a non-smoker with no past medical history and no family history of thrombosis. Her BMI is 23. She had previously been tested for heritable thrombophilias prior to starting the contraceptive pill and was found to be heterozygous for both the Factor V Leiden and the MTHFR gene mutations.
Question 41 (2020)
A 24 year old Greek man is admitted with a new diagnosis of acute lymphoblastic leukaemia. He receives intravenous rasburicase for prevention of tumour lysis syndrome. Three days later he reports breathlessness, with a marked drop in haemoglobin from 120g/l to 60g/l. Blood film examination reveals polychromasia and red cell anisopoikilocytosis with multiple bite cells and blister cells present.
Which of the following is a function of glucose-6-phosphate dehydrogenase (G6PD)?
a. Reduces nicotinamide adenine dinucleotide phosphate (NADP) to NADPH
b. Converts glucose to glucose-6-phosphate (G6P)
c. Converts 6-phosphogluconate (6PG) to pentose
d. Reduces oxidised glutathione (GSSG) to reduced L-glutathione (GSH)
e. Oxidises dihydronicotinamide adenine dinucleotide phosphate (NADPH) to NADP
question 42 (2020)
The finding of the T315I gene mutation in a patient with newly diagnosed chronic myeloid leukaemia is an indication for treatment with which of the following tyrosine kinase inhibitors?
a. Imatinib
b. Ponatinib
c. Dasatinib
d. Nilotinib
e. Bosutinib
Question 43 (2020)
With regard to risk stratification in adult acute lymphoblastic leukaemia (ALL), record whether each of the following statements is true or false:
a. Taking more than four weeks to achieve complete remission is an indicator of high risk
b. The presence of t(9;22) (BCR-ABL1) is an indicator of high risk
c. The presence of t(1;19) (PBX-E2A) is an indicator of standard risk
d. The presence of a hyperdiploidy karyotype is an indicator of high risk
e. A total white cell count of >30x10e9/l in B-Cell ALL is an indicator of high risk
Question 44 (2020)
A 62-year-old woman presents to her GP with an eight-week history of night sweats and increasing lethargy. She first noticed a lump in the right side of her neck a month ago and thinks this is slowly enlarging. On examination additional small lymph nodes can be felt in the axilla and groins bilaterally. WBC 11 x10e9/l, Hb 110 g/l, platelets 100 x10e9/l, lymphocytes 8 x10e9/l. Flow cytometry of the peripheral blood lymphocytes shows them to be CD19+, CD10+, BCL2+, CD5- and CD43-. Core biopsy of the lymph node demonstrates replacement of normal architecture with closely packed, uniform neoplastic follicles. Which of the following chromosomal translocations would you most expect to find in this case?
a. t(11;14)
b. t(11;18)
c. t (8;14)
d. t (3;14)
e. t(14;18)
Question 45 (2020)
For each of the following laboratory assays, select which test is being described from the list below. Each test may be used once, more than once or not at all.
a. Activated Clotting Time (ACT)
b. Activated Partial Thromboplastin Time (APTT)
c. Chromogenic Factor Assay
d. Clauss Fibrinogen
e. Ecarin Clotting Time
f. Fibrinogen antigen
g. Haemoclot Thrombin Time
h. Prothrombin Time (PT)
i. Thrombin Time (TT)
j. 1-Stage PT-based Factor Assay
k. 2-Stage PT-based Factor Assay
i. Platelet poor plasma is incubated with tissue factor and phospholipid at 37oC. Calcium is added and time until clot formation is recorded.
ii. Fresh whole blood is immediately added to a tube containing an activator, such as kaolin, and the time until clot formation is recorded.
iii. Dilute platelet poor plasma is incubated with phospholipid and an excess of thrombin at 37oC. Calcium is added and time until clot formation is recorded. This time is then interpreted in relation to calibration curves of a reference plasma.
iv. Platelet poor plasma is incubated with phospholipid and an activator, such as kaolin, at 37oC. Calcium is added and time until clot formation is recorded.
v. Platelet poor plasma is mixed with a commercial factor deficient plasma. This mix is then incubated with tissue factor and phospholipid at 37oC. Calcium is added and time until clot formation is recorded. The result is interpreted with comparison to dilutions of control plasma.
Question 46 (2020)
For each of the following side effect profiles, select the tyrosine kinase inhbitor that is most likely to be responsible. Answers may be used once, more than once, or not at all.
a. Bosutinib
b. Dasatinib
c. Imatinib
d. Nilotinib
e. Ponatinib
i. Approximately 5% risk of pleural effusion per year of treatment. Pulmonary hypertension and platelet function defects also reported.
ii. Cardiac abnormalities common, occurring in up to 20% of patients. Hyperglycaemia and pancreatitis also recognised adverse effects.
iii. Diarrhoea very common, occurring in one third of patients. Transaminitis and pancreatitis also reported.
iv. Whilst rash, oedema, muscle cramps, diarrhoea and joint pain are all commonly reported, the overall side effect profile is often considered more tolerable than other tyrosine kinase inhibitors.
question 47 (2020)
For each of the following statements regarding the use of fresh frozen plasma (FFP), record whether the claim is true or false.
a. FFP donated via plasmapheresis contains higher concentrations of factor VIII and Von Willebrand factor than units recovered from whole blood.
b. High titre negative (HTN) group O FFP is an appropriate component to select for a group A recipient.
c. Rh D positive FFP units may be transfused to Rh D negative recipients without the use of anti-D prophylaxis.
d. Recipients born after 1st January 1996 should receive imported plasma products to reduce the risk of transfusion transmitted variant Creutzfeldt-Jakob disease.
e. FFP is not prepared from first time donors.
Question 48 (2020)
A 10 year old boy with sickle cell anaemia is due to start a long term transfusion programme. In advance of this, extended red cell phenotyping is performed and is reported as follows:
In light of his red cell phenotype, which additional testing should be performed?
a. D antigen variant typing
b. H Antigen serology
c. P antigen serology
d. T antigen serology
e. U antigen serology
question 49 (2020)
A 29 year old woman is referred to the haematology clinic by the gynaecology team in advance of a planned polypectomy as she had reported a longstanding history of easy bruising and menorrhagia. She tells you that she has von willebrand disease but has never been worked up for this as she has no trauma or surgical history. You performed a desmopressin (DDAVP) challenge and the results are as follows:
What is the most likely diagnosis?
a. Type 1 Von Willebrand Disease
b. Type 1C Von Willebrand Disease
c. Type 2A Von Willebrand Disease
d. Type 2B Von Willebrand Disease
e. Type 2N Von Willebrand Disease
question 50 (2020)
For each of the following statements regarding the use of irradiated blood components in UK practice, record whether the claim is true or false.
a. Once irradiated, the shelf-life of packed red cell units stored at 4oC remains the same
b. All HLA-selected blood components are irradiated regardless of the indication for transfusion
c. It is not necessary to irradiate frozen packed red cell units
d. Solid organ transplant recipients who have received alemtuzumab for transplant conditioning require irradiated blood components indefinitely
e. The degree of immune suppression of the recipient is the strongest risk factor for the development of transfusion-associated graft versus host disease (TA-GVHD) following blood transfusion.
Question 51 (2020)
For each clinicopathological description, select the most likely diagnosis from the alphabetical list. Each option can be used once, more than once or not at all. All patients are iron replete.
Normal Values:
FBC – Hb 115-165g/l (female), Hb 130-180g/l (male), MCV 80-100fl, MCH 27-32pg
HPLC - HbA2 2-3.5%, HbF <0.8%
a. Alpha+ Homozygous trait
b. Beta Thalassaemia trait
c. Hb E Heterozygous
d. Hb Lepore heterozygous
e. Hereditary persistence of fetal haemoglobin (HPFH)
f. HbH disease
g. HbS/Beta-Thalassaemia
h. HbSC
i. HbSS
j. HbS trait
i. 50-year-old Thai woman, generally fit and well. FBC – Hb 114, MCV 74, MCH 24. HPLC – HbA 67%, HbA2 30%, HbF 0.4%
ii. 38-year-old Greek man, generally fit and well. FBC – Hb 132, MCV 83, MCH 30. HPLC – HbA 54%, HbS 40%, HbA2 3.6%, HbF 0.8%
iii. 35-year-old Indian man with chronic anaemia and recurrent vaso-occlusive crises. FBC – Hb 88, MCV 76, MCH 25. HPLC – HbA 44%, HbS 50%, HbA2 4%, HbF 0.6%.
iv. 25-year-old Taiwanese woman with chronic anaemia, intermittently exacerbated by episodes of haemolysis and jaundice. FBC - Hb 75, MCV 70, MCH 24. Blood film demonstrates Heinz bodies on supravital staining. HPLC – HbA 94%, HbA2 2.5%, HbF 0.5%.
v. 40-year-old Indian man, generally fit and well. FBC – Hb 113, MCV 71, MCH 22. HPLC – HbA 83%, HbA2 12.5%, HbF 2.5%.
Question 52 (2020)
A 71-year-old man presents to the emergency department with fever and jaundice occurring three weeks after returning from a Safari holiday in South Africa. His past medical history includes splenectomy following a road traffic accident thirty years ago. Full blood count shows Hb 98 g/l, MCV 110 fl, Plt 80 x10e9/l, WBC 10 x10e9/l. Blood film examination of the red cells identifies intracellular ring parasites with delicate cytoplasm, double chromatin dots and vacuolation. The malaria rapid diagnostic test (RDT) is negative.
Select the most appropriate next course of action from the options presented:
a. Start artemisinin combination therapy & send sample to reference laboratory for malarial diagnostic PCR
b. Start ivermectin & repeat blood film after 12-24 hours for re-assessment
c. Start liposomal amphotericin B & perform bone marrow biopsy for confirmation of suspected leishmaniasis infection
d. Start atovaquone plus clarithromycin & send sample to reference laboratory for confirmation of suspected babesiosis infection
e. Start supportive care for the fever & repeat blood film after 12-24 hours for malarial re-assessment
Question 53 (2021)
Which one of the following is a myeloma-defining event as described by the International Myeloma Working Group?
a. Biopsy-proven amyloidosis
b. Bone marrow plasma cells >60%
c. Recurrent bacterial infection
d. Symptomatic hyperviscosity
Question 54 (2021)
In myeloma, which two of the following are considered standard risk cytogenetic findings on FISH?
a. t(4;14)
b. t(11;14)
c. t(14;16)
d. t(14;20)
e. 1p-
f. 1q+
g. 17p-
h. Hyperdiploidy
Question 55 (2021)
Which two features were added to the International Staging System for myeloma in 2015 to form the Revised-ISS?
a. Body mass index
b. Cytogenetic risk group
c. Erythrocyte sedimentation rate (ESR)
e. Lactate dehydrogenase (LDH)
f. Serum creatinine
Question 56 (2021)
Which of the following is not one of the criteria for a major provoking factor of venous thromboembolism according to the 2016 International Society of Thrombosis and Haemostasis guidelines?
a. Caesarean section
b. General anaesthetic lasting more than 30 minutes
c. Hospitalisation lasting 3 or more days
d. Ipsilateral leg injury causing reduced mobility for 3 or more days
Question 57 (2021)
What percentage of bone marrow cells within a particular lineage need to show dysplastic morphology to meet the WHO criteria for a diagnosis of MDS?
a. >10%
b. >20%
c. >30%
d. >40%
e. >50%
Question 58 (2021)
Which of the following probably has the highest risk of transformation to a haematological malignancy?
a. ARCH (age-related clonal haematopoiesis)
b. CCUS (clonal cytopenia of undetermined significance)
c. CHIP (clonal haematopoiesis of indeterminate potential)
d. ICUS (idiopathic cytopenia of undetermined significance)
Question 59 (2021)
Which of the following genetic mutations is present in >95% of cases of MDS with ring sideroblasts (MDS-RS)?
a. ASXL1
b. EZH2
c. RUNX1
d. SF3B1
e. TP53
Question 60 (2021)
A 76-year-old man is investigated for symptomatic anaemia. His past medical history includes type 2 diabetes, hypertension and emphysema. Hb 85, Neut 0.9, Plt 140. Examination of the bone marrow aspirate demonstrates marked dyserythropoiesis with 4% myeloid blasts. Karyotyping is reported as 46XY. In addition to supportive care, what would your preferred initial management be for this patient?
a. ATG + Ciclosporin
b. Erythropoietin stimulating agent
c. Lenalidomide (low dose)
d. Referral for allogeneic stem cell transplant
e. None of the above
Question 61 (2021)
For each of the following clinicopathological descriptions, select the explanation most likely to account for the scenario. Each answer may be used once, more than once or not at all.
Normal ranges:
Prothrombin time (PT): 10-13 seconds
Activated partial thromboplastin time (APTT): 24-38 seconds
Thrombin time (TT): 10-16 seconds
Clauss Fibrinogen (FGN): 2-4 g/l
a. Acquired haemophilia A
b. Acquired haemophilia B
c. Apixaban
d. Citrate sample tube overfilled
e. Citrate sample tube underfilled
f. Dabigatran
g. Factor VIII deficiency
h. Factor IX deficiency
i. Factor XI deficiency
j. Factor XII deficiency
k. Factor XIII deficiency
l. Lupus anticoagulant
m. Unfractionated heparin
n. Warfarin
i. A patient attends the pre-operative clinic in preparation for a cholecystectomy. Bloods taken in clinic show PT 8s, APTT 18s, FGN 2.1 g/l. The patient is re-called, re-bled and the repeat tests are as follows: PT 12s, APTT 26s, FGN 2.3 g/l.
ii. An 85 year old woman presents to the emergency department with epistaxis. She has no past history of bleeding disorders. Initial coagulation screen shows PT 14.6s, APTT 180s, FGN 2.2 g/l. A time dependent inhibitor screen is performed, the immediate APTT 50:50 mix is 40 seconds and the 2-hour incubation APTT 50:50 mix is 131 seconds.
iii. A male neonate is born at term without complication. Two days later he develops pronounced bleeding at the site of his umbilical cord stump. PT 12s, APTT 30s, TT 14s.
iv. A 70 year old man is admitted with a fractured neck of femur. PT 11s, APTT 56s, APTT 50:50 mix with normal plasma 35s. The orthopaedic team proceed with surgery and there are no bleeding complications.
v. A 50 year old woman presents to the emergency department with acute upper gastrointestinal bleeding, occurring four weeks after a new diagnosis of deep vein thrombosis. PT 13s, APTT 42s, TT 90s.
question 62 (2021)
A 55 year old woman is due to undergo bowel resection for a recently diagnosed adenocarcinoma. Three months ago she had drug eluting coronary stents inserted following a hospital admission with acute coronoary syndrome. She currently takes aspirin plus clopidogrel. In consultation with surgical and cardiology colleagues what would be your preferred peri-operative management from the list below. Select one option.
a. Continue aspirin. Stop clopidogrel 5 days prior to surgery. Re-start 10 days post-op.
b. Continue aspirin. Stop clopidogrel 5 days prior to surgery. Re-start post-op when haemostasis achieved.
c. No adjustment required. Continue aspirin and clopidogrel.
d. Stop aspirin and clopidogrel 5 days prior to surgery. Re-start 10 days post-op.
e. Stop aspirin and clopidogrel 5 days prior to surgery. Re-start post-op when haemostasis achieved.
question 63 (2021)
Based on their known pharmacokinetics, match each of the following anti-platelet agents to the time from drug administration during which platelet transfusion would be expected to have reduced efficacy. Each answer may be used once, more than once or not at all.
a. 1 hour
b. 2 hours
c. 4-6 hours
d. 12 hours
e. 16-18 hours
f. 18-26 hours
g. 24-48 hours
h. 5-7 days
i. Abciximab
ii. Aspirin
iii. Clopidogrel
iv. Prasugrel
v. Ticagrelor
question 64 (2021)
A viscoelastic assay is performed during the management of a post-partum haemorrhage. Based on the following three thromboelastography traces, select the treatment you would be most likely to prioritise administering next. Each answer may be used once, more than once or not at all.
a. Cryoprecipitate
b. Fibrinogen concentrate
c. Fresh frozen plasma
d. Packed red cells
e. Platelets
f. Tranexamic acid
question 65 (2022)
The MYD88 L265P mutation is present in most cases of Waldenstrom Macroglobulinaemia. Which of the following is the next most frequently occurring mutation in this condition?
a. ASXL1
b. CXCR4
c. BRAF V600E
d. TET2
e. TP53
question 66 (2022)
Your patient has been on ruxolitnib for primary myelofibrosis but over the last 3 months has developed new constituional symptoms alongside early satiety and left upper quadrant discomfort. You plan to change the ruxolotinib to fedratinib, an alternative JAK2 inhibitor. Which of the following deficiencies must specifically be assessed and corrected prior to commencing treatment with fedratinib?
a. Copper
b. Vitamin B1
c. Vitamin B12
d. Selenium
e. Zinc
Question 67 (2022) (HARD!)
For each of the following clinicopathological descriptions, select the diagnosis that best fits the scenario. Each answer may be used once, more than once or not at all.
a. AESOP Syndrome
b. CANOMAD Syndrome
c. Monoclonal gammopathy of uncertain significance
d. Plasma cell myeloma
e. POEMS Syndrome
f. Schnitzler Syndrome
g. Solitary bone plasmacytoma
h. Solitary extramedullary plasmacytoma
i. TEMPI Syndrome
j. None of the above
Normal Ranges: Hb 130-150, MCV 80-90, WBC 4-10, Plt 150-400, Erythropoietin 2-18, Cr 50-100, Adj Ca 2-2.5, Total protein 60-80, Kappa LC 2-20, Lambda LC 2-20
i. A 43-year-old man presents to hospital with a one year history of weight loss and numbness in his feet. Three months prior to admission the peripheral neuropathy become ascending and progressive. On examination there was decreased sensation distally in all four limbs with loss of deep tendon reflexes. He was also noted to have hyperpigmented, thickened skin on both legs. His liver and spleen were enlarged. Bloods showed an IgG paraprotein of 3g/l. X-ray skeletal survey revealed an osteosclerotic lesion in the right femur.
ii. A 66-year-old man is referred to the dermatology clinic for evaluation of an extensive thoracic skin patch present for the previous one year. On examination there was a 10cm red-brown shiny patch below the right breast. Normal full blood count. IgG paraprotein 2g/l. Skin biopsy demonstrated diffuse interstitial vascular hyperplasia. Chest X-ray revealed an osteodense, expansile lesion within the sixth rib.
iii. A 47-year-old man presents with two years of left lower back pain. MRI imaging reveals a left side perinephric fluid collection and abdominal ascites. On further examination nail telangectasia was noted. His past medical history includes a diagnosis of polycythaemia vera, for which he is venesected regularly. Hb 126, Erythropoietin 4500, IgG paraprotein 10g/l.
iv. A 65-year-old man presents to the emergency department with drowsiness and nausea. Her family report reduced fluid intake over the last five days. The patient complains of hip pain but is otherwise unable to provide a history. On examination she is mildy confused and drowsy (GCS 14/15). Hb 100, WBC 4.5, Plt 200, Creatinine 350, Adj Calcium 3.75, Total protein 110.
v. A 75-year-old man presents to his GP with lethargy and right hip pain, present for the last 6-12 months. His past medical history includes heart failure, emphysema, chronic kidney disease and bilateral hip replacements. On examination he appears pale and is breathless from walking across the car park. Hb 110, MCV 82, WBC 4.5, Plt 250, Cr 150, Adj Ca 2.3, IgG paraprotein 2g/l, Kappa LC 100, Lambda LC 50, Ratio 2.
Question 68 (2022) (HARD!)
A 76-year-old man presents to the rheumatology clinic with a history of symmetric polyarthritis of his large and small joints. This was accompanied by intermittent low grade fevers and recurrent urticarial rash.
Hb 100, MCV 110, WBC 5, Plt 200, CRP 280, Ferritin 1100
A subsequent bone marrow biopsy demonstrated marked trilineage dysplasia with vacuolated myeloid cells. A myeloid NGS panel is performed. A mutation in which one of the following genes would best account for this patient’s presentation?
a. ETV6
b. IDH2
c. SFS3B1
d. TP53
e. UBA1
Question 69 (2022)
For each of the following sites of extranodal marginal zone lymphoma (ENMZL), select the infectious organism most likely to precipitate it. Each answer may be used once, more than once or not at all.
a. Borrelia
b. Campylobacter
c. Chlamydia psittaci
d. Escherichia coli
e. Helicobacter pylori
f. Herpes Simplex Virus
g. Norovirus
h. Salmonella
i. Staphylococcus aureus
j. None of the above
i. Gastric ENMZL
ii. Ocular ENMZL
iii. Small intestine ENMZL
iv. Skin ENMZL
v. Breast ENMZL
Question 70 (2022)
For each of the following statements regarding the laboratory diagnosis of malaria in the UK, state whether it is true or false.
a. The parasitaemia count should be made from the examination of 200 high power fields
b. Geimsa staining is appropriate for use in preparation of both thick and thin films
c. Red cells infected with P. falciparum are typically enlarged in size
d. A negative malarial immunochromatographic test (RDT) also excludes a diagnosis of babesiosis
e. All slides prepared for examination of malaria must be dual reported
Question 71 (2022)
Which of the following best describes the mechanism of action for Venetoclax
a. Induction of BCL2. Underexpression of BCL2 reduces production of the anti-apoptotic protein BAX. Inducing BCL2 expression increases BAX levels and leads to cell death.
b. Inhibition of BCL2. Overexpression of BCL2 increases production of the anti-apoptotic protein BAX. Inhibiting BCL2 reduces BAX levels and leads to cell death.
c. Inhibition of BCL2. Overexpression of BCL2 sequesters the pro-apoptotic protein BAX. Inhibiting BCL2 releases BAX and leads to cell death.
d. Induction of Bruton Tyrosine Kinase (BTK). Induction of BTK inhibits NFkB DNA binding leading to reduced cell migration, proliferation and survival.
e. Inhibition of Bruton Tyrosine Kinase (BTK). Inhibition of BTK inhibits NFkB DNA binding leading to reduced cell migration, proliferation and survival.
Question 72 (2023)
A 25 year old woman who had immune thrombocytopenia (ITP) one year ago attends clinic and asks you about family planning. Select true or false for each of the following statements regarding pregnancy in women with a previous diagnosis of ITP.
a. ITP is more likely to relapse during pregnancy
b. The risk of neonatal ITP (NITP) is higher in neonates if an older sibling had NITP
c. The risk of neonatal ITP is higher in women who have a platelet count <50 x10e9/l within 3 months of delivery
d. Women with ITP who relapse during pregnancy are less likely to have severe thrombocytopenia (<30 x10e9/l) than women who relapse outside of pregnancy
e. Women with ITP who relapse during pregnancy are more likely to require a change in their ITP treatment than women who relapse outside of pregnancy
question 73 (2023)
Polatuzimab vedotin is licensed for the treatment of high grade B cell lymphomas. Select the following statement which best describes the mechanism of action for this drug.
a. Antibody-drug conjugate. The antibody targets CD79a to deliver a microtubule polymerisation inhibitor.
b. Antibody-drug conjugate. The antibody targets CD79b to deliver a microtubule polymerisation inhibitor.
c. Antibody-drug conjugate. The antibody targets CD20 to deliver a microtubule polymerisation inhibitor.
d. Antibody-drug conjugate. The antibody targets CD79a to deliver a topoisomerase inhibitor.
e. Antibody-drug conjugate. The antibody targets CD20 to deliver a topoisomerase inhibitor.
question 74 (2023)
Select which one of the following patients diagnosed with diffuse large B cell lymphoma has the highest risk of central nervous system (CNS) relapse.
a. 40 year old. ECOG performance status 0. Stage IV disease. Elevated LDH. Lung and kidney involvement
b. 51 year old. ECOG performance status 3. Stage III disease. Elevated LDH. No extranodal involvement
c. 65 year old. ECOG performance status 1. Stage IV disease. Elevated LDH. Liver and testicular involvement
d. 72 year old. ECOG performance status 3. Stage III disease. Elevated LDH. No extranodal involvement
e. 80 year old. ECOG performance status 3. Stage IV disease. Normal LDH. Lung involvement
Question 75 (2023)
Of the available options, select which one of the following genes is most strongly associated with a germline predisposition for leukaemia.
a. CXCR4
b. DDX41
c. ETV6
d. RUNX1
e. TP53
Question 76 (2023)
A 36 year old man presents to his GP with a two month history of blurred vision and exopthalmos. His past medical history includes chronic joint pain, present for the last four year and primarily affecting his knees and elbows. He also reported intermittent generalised itching. Full blood count, renal and liver function tests were normal. CRP and ESR mildly elevated. MRI brain identified a pituitary lesion and abnormal retrobulbar soft tissue infiltration. Body imaging demonstrated bilateral peri-nephric stranding and sclerotic bone changes in the distal portions of all long bones.
A bone biopsy of the left tibia was performed. Which one of the following genetic abnormalities is most likely to be present in this condition?
a. BRAF V600
b. CSF3R T618I
c. JAK2 V617F
d. KIT D816V
e. MYD88
Question 77 (2023)
A 65 year old woman attends the haematology clinic for her three mothly review of ibrutinib. She has been taking ibrunitib for the last two years for chronic lymphocytic leukaemia, which remains in a very good partial remission. She reports that her home blood pressure monitor has consistently recorded high levels over the last two months, ranging from 140/90 to 160/100. This is confirmed when repeated in clinic today. She has no previous hypertension or other cardiovascular history.
Select your preferred initial management strategy from the list below:
a. Start diltiazem and continue ibrutinib at current dose
b. Start diltiazem and reduce ibrutinib dose by 140mg
c. Start ramipril and continue ibrutinib at current dose
d. Start ramipril and reduce ibrutinib dose by 140mg
e. Stop ibrutinib and refer patient for cardiology assessment
Question 78 (2023)
Select which one of the following statements is best describes the pathophysiology of paroxysmal nocturnal haemoglobinuria (PNH).
a. A gain-of-function PIG-A gene mutation results in increased presence of CD31 and CD36 proteins. CD31 promotes the action of C5 convertases on red cells, rendering them vulnerable to the membrane attack complex (MAC). This results in chronic red cell haemolysis.
b. A gain-of-function PIG-A gene mutation results in increased presence of CD55 and CD59 proteins. CD55 promotes the action of C3 convertases on red cells, rendering them vulnerable to the membrane attack complex (MAC). This results in chronic red cell haemolysis.
c. An inactivating PIG-A gene mutation results in deficiency of CD31 and CD36 proteins. CD31 blocks the formation of C3 convertases, and so CD31 deficiency renders red cells vulnerable to the membrane attack complex (MAC). This results in chronic red cell haemolysis.
d. An inactivating PIG-A gene mutation results in deficiency of CD55 and CD59 proteins. CD55 blocks the formation of C3 convertases, and so CD55 deficiency renders red cells vulnerable to the membrane attack complex (MAC). This results in chronic red cell haemolysis.
e. An inactivating PIG-A gene mutation results in deficiency of CD71 and CD235a proteins. CD71 blocks the formation of C3 convertases, and so CD71 deficiency renders red cells vulnerable to the membrane attack complex (MAC). This results in chronic red cell haemolysis.
question 79 (2023)
Select which one of the following cytogenetic findings is most associated with a poor rate of response to H. pylori eradication in the treatment of gastric mucosa-associated lymphoid tissue (MALT) lymphoma.
a. t(1;14) / IGH::BCL10
b. t(3;14) / IGH::FOXP1
c. t(11;18) / BIRC3::MALT1
d. t(14;18) / IGH::MALT1
e. Trisomy 3