HaemBase

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question 1

i – D. Hermansky-Pudlak

ii – H. Pseudo-Type 2 VwD

iii – B. Glanzman Thromboasthenia

(detail)

question 2

i – J. Bernard-Soulier

ii – R. Paris-Trousseau-Jacobsen Syndrome

iii – Y. Wiskott-Aldrich

iv– N. Grey Platelet Syndrome

v – M. Glanzman Thrombasthenia

(detail)

question 3

i - C. The t(2;5) NPM1-ALK fusion gene is seen in ALK+ Anaplastic large cell lymphoma and confers a better prognosis than ALK- subtypes.

ii - F. The t(11;14) present in Mantle Cell lymphoma leads to overexpression of Cyclin D1

iii - H. inv(16) is often associated with a notable eosinophilia in AML. The crystals described in the case are Charcot-Layden crystals.

iv - D. t(3;14) FOXP1-IgH is seen in 20% of orbital marginal zone lymphomas.

v - G. Other translocations listed here may be seen in ALL, but t(12;21) would be most typical for B-ALL in children aged around 10 years.

question 4

Correct Answer = B

(detail)

question 5

Correct Answer = C

(detail)

question 6

Correct Answer = E

(detail)

question 7

i - B. Hereditary Elliptocytosis

ii - G. Familial Pseudohyperkalaemia

iii - A. Hereditary Spherocytosis

iv - C. Hereditary Pyropoikilocytosis

v - H. South East Asian Ovalcytosis

(detail)

question 8

i - C

ii - F

iii - A

iv - E

v - B

(detail)

question 9

Correct answer = B (detail)

question 10

Correct answer = E (detail)

question 11

Correct answer = E (detail)

question 12

i - E

ii - J

iii - A

iv - B

v - D

(details here, here and here)

question 13

Correct answers = A & D (rationale)

question 14

i - D. Liposomal daunorubicin + cytarabine has a NICE recommendation for patients with therapy-related AML or AML with MDS-related change. The liposomal preparation is thought to better home to the marrow and have a prolonged half-life whilst there. This comes at a cost of a longer time to count recovery than standard DA.

ii - G. Azacitidine has a NICE recommendation for AML with 20-30% blasts. It can provide disease control, improving quality and length of life, but is not curative. It is given as a subcutaneous injection in an outpatient setting.

iii - E. Midostaurin is a FLT3 inhibitor and has a NICE recommendation for AML with FLT3-ITD mutation detected. It is given for 14 days starting on day 8 alongside standard DA.

iv - B. Gemtuzumab Ozogamicin is an anti-CD33 antibody combined with a cytotoxic antibiotic. It has a NICE recommendation for AML with CD33+ immunophenotype and good or intermediate cytogenetic risk (or unknown risk if cytogenetics fails).

(details here. N.B. This question was written in Jan 2019 based on current NICE recommendations.)

question 15

i – F. Classically presents with epistaxis / nasal congestion / facial oedema but other extranodal sites not infrequently involved. NK cells usually CD56+.

ii – J.

iii – B. This is the typical immunophenotype of a normal follicular T-helper cell, thought to be the cell of origin for AITL

iv – C. Strong CD30+ is a typical feature.

v – H. History most useful clue here.

(more info here and here.)

Question 16

i. B. Primary warm AIHA antibodies are usually panreactive, but in 3% of cases specificity can be assigned (Anti-c, e & E most common). As this is autoimmune, the antibody must be directed against one of the patient’s own red cell antigens. The R1R1 nomenclature tells us that his Rh phenotype is C, D, e.

ii. E. Patients with a low-level cold agglutinin are likely to be asymptomatic, and only be discovered incidentally.

iii. F. This is the Donath-Landsteiner antibody, responsible for paroxysmal cold haemoglobinuria.

iv. D. The description is of cold haemagglutinin disease (CHAD). 90% of cases are due to an Anti-I antibody.

(Details: see AIHA) 

question 17

Correct Answer = E.

Across two case series of drug-induced immune haemolysis (DIHA) with a total of 188 patients, ceftriaxone was thought to be the causative agent in 20 cases (Tazocin in 17, Diclofenac in 15 and fludarabine in 6). >130 drugs have been implicated in DIHA.

question 18

Correct Answer = D.

Median OS, in years, as follows: CALR - 17.7, JAK2 - 9.2, MPL - 9.1, Triple Neg - 3.2. Reference

Patients with triple negative myelofibrosis are more likely to have High Molecular Risk (HMR) mutations - IDH1/2, ASXL1, EZH2, SRSF2.

question 19

Correct Answer = D

See here

Question 20

Correct Answer = E

See here

question 21

i. C. This is minor incompatibility. Guidelines recommend use of donor ABO group.

ii. A. This is minor incompatibility. Guidelines recommend use of Group A, high titre negative platelets. Rh D negative given until patient starts typing as Rh D positive.

iii. G. This was a major incompatibility at the time of transplant but the patient has now fully converted to the donor blood group and so donor-compatible units should be used.

iv. F. This is major incompatibility. Guidelines recommend use of Group B, high titre negative platelets.

N.B. Many hospitals take a more pragmatic approach and simply use group O red cells for all allograft patients.

See here

question 22

Correct Answer = B

See here

question 23

Correct Answer = A

See here and here

Question 24

Correct Answer = C

See here

Question 25

Correct Answer = C

See here

Question 26

i. G. Fanconi.

ii. C. CAT.

iii. E. Dyskeratosis.

iv. B. Aplastic Anaemia

v. A. Anorexia

See here

Question 27

i. J. Schwachman-Diamond.

ii. I. MonoMAC

iii. H. Hypoplastic MDS

iv. F. Emberger Syndrome

v. D. Diamond-Blackfan

See here

Question 28A

Correct Answer = E

See here

Question 28B

Correct Answer = A

See here

Question 29

Correct Answer = D

The HIT probability score in this case is 6 (high probability). The patient should be switched to an alternative anticoagulant immediately whilst awaiting outcome of HIT investigations. Warfarin should not be started until after a minimum of 5 days anticoagulation with an alternative drug.

See here

Question 30

i. D

ii. G

iii. C

iv. B

See here

Question 31

i. D.

ii. H.

iii. A.

iv. F.

v. J.

See here

(References for case studies - https://www.sciencedirect.com/science/article/pii/S0001706X14000369 & https://jamanetwork.com/journals/jamadermatology/fullarticle/407845)

Question 32

Correct Answer = E.

t(8;14) is present in >80% of Burkitt cases. Other MYC rearrangements are less commonly present, such as t(8;22) and t(2;8).

See here

Question 33

Correct Answer = B.

This is Gaucher’s Disease. A white cell enzyme screen will identify the low acid glucocerebrosidase activity diagnostic of the condition.

See here

Question 34

Correct Answer = A

Pregnant women presenting with a Hb <110g/l at booking, without a known history of haemoglobinopathy, should be advised a short trial of oral iron supplementation. There is no need to check serum ferritin first.

See here

Question 35

Correct Answer = D

Ref: Gehrie & Tourmey 2014

Question 36

Correct Answer = D

Ref: Gehrie & Tourmey 2014

Question 37

Correct Answer = A

See here under the Haemphilia B sub-heading

Question 38

i. B. Dabigatran

ii. E. Warfarin

iii. C. Dateparin

iv. D. Rivaroxaban

v. A. Abciximab

See here

Question 39

a. True

b. False

c. Flase

d. True

e. False

See here

Question 40

i. G

ii. C

iii. F

iv. A

v. A

See here. There has previously been a concern that MTHFR gene mutations might be associated with an increased thrombotic risk. That has since been shown not to be the case, but it continues to be tested in some clinics, particularly outside the UK.

Question 41

Correct Answer = A

See here.

question 42

Correct Answer = B

See here.

Question 43

a. True

b. True

c. False

d. False

e. True

See here

Question 44

Correct Answer = E. Diagnosis is follicular lymphoma.

See here.

question 45

i. H. Prothrombin Time

ii. A. Activated Clotting Time

iii. D. Clauss Fibrinogen

iv. B. APTT

v. J. 1-Stage PT-based Factor Assay

See here.

Question 46

i. B. Dasatinib

ii. D. Nilotinib

iii. A. Bosutinib

iv. C. Imatinib

See here

Question 47

i. False

ii. False

iii. True

iv. False

v. True

See here

question 48

Correct Answer = E.

This one was hard! (I think). See here for info on U antigen.

question 49

Correct Answer = B. Type 1C Von Willebrand. Also known as Vicenza

The DDAVP challenge shows a very short half-life of the VWF, typical of 1c/Vicenza. Normal VWF half-life should be more in the order of 8 hours. See VWD overview here.

question 50

a. False. The unit must be transfused within 14 days of irradiation, or the end of its pre-existing shelf-life, whichever is shorter.

b. True. The degree of HLA mismatch between donor and recipient is the strongest risk factor for the development of TA-GVHD.

c. True

d. False. Irradiation is only recommended for recipients of alemtuzumab with they also have a haematological diagnosis.

e. False. See answer B.

question 51

i. C. HbE Heterozygous

ii. J. HbS Trait

iii. G. HbS/Beta-Thalassaemia

iv. F. HbH Disease

v. D. Hb Lepore heterozygous

question 52

Correct Answer = D.

Babesiosis. Morphology of babesiosis can be very similar to falciparum malaria but the rapid diagnostic test will be negative. Babesiosis infection is often asymptomatic but older age, asplenism, chronic liver or kidney disease and immunosuppressed states all increase the risk of severe infection. See more on CDC website.

Question 53

Correct answer = B. BM plasma cells >60%

Question 54

Correct answer = B & H. t(11;14) and Hyperdiploidy

Question 55

Correct answer = B & E. Cytogenetic risk group and LDH

question 56

Correct answer = D. Leg injury causing reduced mobility for 3 or more days is one of the criteria for a minor provoking factor of VTE. See more here.

Question 57

Correct answer = A. >10%

Question 58

Correct answer = B. CCUS. Early evidence suggests majority of patients will develop MDS with follow-up (https://pubmed.ncbi.nlm.nih.gov/28424163/)

Question 59

Correct answer = D. SF3B1

Question 60

Correct answer = B. ESA. This patient has low risk MDS and symptomatic anaemia. Age and PMH make it less likely that intensive treatment is advisable and although this could always be considered, ESA would be the preferred initial treatment. Low dose lenalidomide is indicated for cases of 5q- Syndrome.

Question 61

i. E. Citrate sample tube underfilled. This leads to a relatively increased concentration of calcium in the assay, shortening the clotting times.

ii. A. Acquired haemophilia A. FVIII inhibitors are typically time dependent, compared to immediate action of FIX inhibitors.

iii. K. Factor XIII deficiency. This is a typical presentation of this rare inherited factor deficiency.

iv. J. Factor XII deficiency. Does not cause a bleeding phenotype.

v. F. Dabigatran. TT very sensitive to the presence of direct thrombin inhibitors. PT normal in 30% of patients on dabigatran.

Question 62

Correct Answer = B.

Based on ESC guidelines 2017. Patient is in moderate thrombotic risk category (1-12 months since stent placement). Surgery cannot be deferred due to cancer. Surgery is not neuro or prostate and so aspirin can generally be continued. Even following recent ACS clopidogrel should be held for major surgery that cannot be deferred.

question 63

i. Abciximab - A. 1 hour

ii. Aspirin (uncoated tablets) - B. 2 hours

iii. Clopidogrel - D. 12 hours

iv. Prasugrel - E. 16-18 hours

v. Ticagrelor F. 18-26 hours

(N.B. Bit spooky, time gets longer alphabetically…)

question 64

i. C. FFP

ii. E. Platelets

iii. F. Tranexamic Acid

question 65

B. CXCR4. Present in approx. 30% of WM cases.

More here.

question 66

B. Vitamin B1 (Thiamine). Cases of Wernicke’s encephalopathy causes early termination of initial fedratinib trials. Thought now due to underlying poor nutritional state of patients entering the trial but nevertheless is now a required part of pre-reatment workup.

More here.

Question 67

i. E. POEMS.

ii. A. AESOP

iii. I. TEMPI

iv. D. Plasma cell myeloma

v. C. MGUS

Case reports used:

POEMS – Korean Medical Science 1992

AESOP – Medicine 2003 (Patient went on to develop POEMS, a known association)

TEMPI – Internal Medicine Japan 2020

Question 68

E. UBA1 gene mutation. This is VEXAS Syndrome.

Case report used - Frontiers in Immunology 2021

Question 69

i. E. H. pylori

ii. C. Chlamydia

iii. B. Campylobacter

iv. A. Borrelia

v. J. None of the above

Question 70

a. True.

b. True.

c. False.

d. False.

e. True.

Question 71

Correct Answer = C

Question 72

a. False

b. True

c. True

d. False

e. True

This information is based on this helpful Blood 2023 paper.

question 73

Correct Answer = B

Question 74

Correct Answer = C.

Patients C, D and E all score 4 points on the CNS-IPI. Although not specified in the CNS-IPI, testicular is now recognised as high risk extra-nodal site (BSH guideline 2020) that I think most MDTs would agree places Patient C above D&E.

(Patients A & B have CNS-IPI scores of 3).

Question 75

Correct Answer = B.

See Table 3 of ELN 2022 AML guideline

Question 76

Correct Answer = A. BRAF V600

The description is of Erdheim Chester Disease. Case Report Ref: Erdheim-Chester Disease: Case Report with Aggressive Multisystem Manifestations and Review of the Literature | Case Reports in Oncology | Karger Publishers

Question 77

Correct Answer = C.

Answer based on BSH Good Practice Paper 2021, which specifically recommends avoiding BTKi dose modifications and treating the hypertension. Avoid diltiazem due to drug interactions.

Question 78

Correct Answer = D

N.B

CD31 = Glycoprotein 1a (a platelet membrane protein)

CD36 = Platelet glycoprotein 4 (found on a range of mature blood cells)

CD55 = Decay-accelerating factor (DAF)

CD59 = MAC-inhibitory protein (MAC-IP)

CD71 = Transferrin receptor

CD235a = Glycophorin A

question 79

Correct Answer = C. t(11;18).

Reference: Gastroenterology 2002