If possible find a friendly consultant who will mark your answer and provide some feedback. I have tried to give some suggested answers to each question but I am not an examiner and they will be multiple approaches I have not considered. Many of them better than what I have written here!
Try to use the first 3-5 minutes to map out the essay before you start, hopefully this way you won’t be crossing out and drawing arrows all over the finished piece.
Whilst reading the question, think about what the examiners are wanting to see in your answer, e.g.:
- They ask for a letter so give them a letter
- Your practical experience/knowledge of preparing a patient for chemotherapy
- Ability to communicate clearly with other healthcare professionals
- Knowledge of the principles for 1st line treatment of Mantle cell lymphoma
- Bonus: Ability to name and discuss relevant clinical trials
Example answer plan:
1. 24-hour urine collection for creatinine clearance
3. Bloods inc. FBC, U&E, LFT, Bone, LDH, Hep B, Hep C & HIV
4. Patient information leaflets on Mantle Cell Lymphoma and R-DHAP given to patient
5. Clinic in one week to consent for chemotherapy and book treatment start date
Stage 4 Mantle Cell Lymphoma
Performance Status 1
Medications started today:
Allopurinol 300mg OD
Today’s blood results awaited
Body of letter
- Re-cap patient’s history
- Diagnosis given to patient – mantle cell lymphoma, patient’s presentation typical of the disease, indolent or aggressive course, treatable but not curable, median survival 4-5 years in all comers
- Treatment options – he is a fit patient with stage IV disease and so the goal is chemotherapy followed by autograft, then rituximab maintenance. Treatment is cytarabine-based and a current common regimen is R-Maxi-CHOP/Cytarabine (NORDIC protocol). Bonus: LyMa trial 2017 recently published showing R-DHAP also an effective option. LyMa also demonstrated the benefit of R-maintenance post autograft.
- Risks – neutropenic sepsis (Bonus: mention 24 hr emergency contact card), cytopenias, GI, infusion reactions, renal impairment, iritis, cardiac impairment, tumour lysis syndrome, steroid side effects, hair loss, impaired fertility, treatment-related mortality (The MacMillan website is a great place to look for regimen specific side effects).
- Describe the management plan given above and the rationale for it.
Example answer plan:
CLL (Binet Stage C).
CLL Score at least 4 (surface Ig not given)
Consider the need to rule out high grade transformation - is there a site of particular bulk that should be biopsied to ensure still CLL.
TP53 loss / mutation must be looked for (FISH/Molecular) as will direct treatment choices
Patient is anaemic, look for haemolysis - film, reticulocyte count, bilirubin, haptolgobins, DAT
Off trial, assuming no TP53 disruption - chemoimmunotherapy.
Give your preference and rationale (e.g. FCR based on CLL10 trial)
Irradiated blood products for life - purine anologues, bendamustine
consider prophylactic LMWH - is there venous compression 2o to the pelvic disease
Annual ‘flu and pneumococcal vaccination
Patient Support Group,
Re-Stage +/- Re-biopsy
Avoid chemoimmunotherapy as remission only 2 years
Consider ibrutinib or R-idelasilib, based on availability and tailored to patient characteristics.
Example Answer Plan
The probably diagnosis here is TTP
Provide table with a differential diagnosis (other causes of MAHA)
Send ADAMTS13 level (<5% diagnostic) but don’t wait for result before proceding with planning treatment
Haemolysis markers - LDH, retic, bilirubin, haptoglobins
DAT - expected to be normal
Troponin - poor prognostic sign if elevated
Coagulation screen - expected to be normal, aids in excluding differentials
Other Tests (for excluding differentials) - HIV, Hep B, Hep C, TFT, Autoantibodies, CT/MRI brain, tumour markers, CT CAP
Management over next 24 hours:
Multi-disciplinary team-working required
Plasma Exchange with SD-FFP within 4-8 hours.
Removes anitbody + ULMW VWF multimers
SD-FFP reduces risk of TTI and adverse immune responses.
If PEX not available within 4-8 hours, transfuse SD-FFP
e.g. 1mg/kg meythlprednisolone
Stops antibody production
Anti-VWF antibody, inhibits interactions between VWF and platelets
NEJM 2019 - shorter time to normal platelet count, lower composite mortality
ITU / high dependency
PPI, folic acid
Red cell transfusion if required
Who will need this service (paient population)?
Who will you need on the team?
How will the service be validated / accredited?
What will be needed pre/during/post-procedure for good patient care
See here for some answers to these questions
Example Answer Plan
Stop transfusion, maintain IV access with saline
Assess ABC and call for help - e.g. ITU support / crash team
Check patient identity against blood component being transfused. Examine unit for discolouration / clumps.
This is a severe acute transfusion reaction (i.e. Shock is present & this is not in keeping with patient’s underlying condition)
Differential: ABO incompatibility, Anaphylaxis, Bacterial contamination of red cell unit
FBC - ?change in haemoglobin level vs pre-transfusion
U&E - ?acute renal failure
LFT/Bilirubin - ?haemolysis
PT/APTT/FGN - ?DIC
Group & Screen - for comparison to previous samples
Direct antigloublin test
Urine - ?haemoglobinuria
Return blood unit to transfusion lab for confirmation of typing / in case of further testing for bacterial contamination
Chest X-ray - ?ARDS/Pul oedema/TACO/TRALI
(For review at later date:
Tryptase, IgA level, haptoglobins)
?ABO incompatibility - Intensive supportive care, dialysis, involvement of renal team
?Anaphylaxis - IM adrenaline 0.5ml of 1:1000
?Bacterial Contamination - broad spectrum IV antibiotics, contact NHSBT to consider component recall
Root Cause Analysis
Consider all steps in the ‘vein-to-vein’ pathway (2005 Blood Safety and Quality Regulations)
Patient ID minimum requirements
Documentation - prescription, request form
Sample handling & labelling
Laboratory systems - electronic data management, lab analysers
SHOT report 2017
1 ABO-incompatible red cell transfusion, 342 near misses, majority wrong blood in tube
Issues around ABO-incompatibility formed 2 of the 3 key recommendations for that year
Duty of candour to patient to explain why re-testing
?Need for new work flows/systems in the day unit
Two sample policy - this case an example of its utility.
Report to SHOT haemovigilance scheme