intro

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!

question 1

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:

 

Dear GP,

 

Management Plan:

1. 24-hour urine collection for creatinine clearance

2. ECG

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

 

Diagnosis:

Stage 4 Mantle Cell Lymphoma

Performance Status 1

 

Medications started today:

Allopurinol 300mg OD

 

Results:

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.


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question 2

Example answer plan:


Diagnosis

  • CLL (Binet Stage C).

  • CLL Score at least 4 (surface Ig not given)

Further work-up:

  • 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

Treatment:

  • Offer trial

  • Off trial, assuming no TP53 disruption - chemoimmunotherapy.

  • Give your preference and rationale (e.g. FCR based on CLL10 trial)

Picture1.png

Supportive Care:

  • 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,

Relapse:

  • Re-Stage +/- Re-biopsy

  • Avoid chemoimmunotherapy as remission only 2 years

  • Consider ibrutinib or R-idelasilib, based on availability and tailored to patient characteristics.

Picture2.png

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question 3

Example Answer Plan

Diagnosis:

  • The probably diagnosis here is TTP

  • Provide table with a differential diagnosis (other causes of MAHA)

Invesitgation:

  • 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:

  • Medical emergency

  • Multi-disciplinary team-working required

  • Plasma Exchange with SD-FFP within 4-8 hours.

    • Removes anitbody + ULMW VWF multimers

    • Replaces ADAMTS13

    • SD-FFP reduces risk of TTI and adverse immune responses.

    • If PEX not available within 4-8 hours, transfuse SD-FFP

  • Steroids

    • e.g. 1mg/kg meythlprednisolone

    • Stops antibody production

  • Caplacizumab

    • Anti-VWF antibody, inhibits interactions between VWF and platelets

    • NEJM 2019 - shorter time to normal platelet count, lower composite mortality

  • Supportive Care

    • ITU / high dependency

    • PPI, folic acid

    • Red cell transfusion if required

Apheresis Service

  • 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

Question 4

Example Answer Plan

A

Immediate Actions:

  • 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.

Diagnosis:

  • 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

Investigation:

  • 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

  • Blood cultures

  • 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)

Further management:

  • ?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

B

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

Corrective Action

  • Re-sample patient

  • Duty of candour to patient to explain why re-testing

Preventative Action

  • Staff education

  • ?Need for new work flows/systems in the day unit

  • Two sample policy - this case an example of its utility.

External Reporting

  • Report to SHOT haemovigilance scheme