Haematology

Question 1

Approach to work up of anaemia?

Answer 1

Firstly evaluate MCV and look at blood film for any obvious abnormalities / dysplasia

Low MCV

• Iron deficiency -> iron studies
• Chronic disease -> Can check soluble transferrin receptor, which will be high in IDA
• Thalassaemia (normal iron studies, blood film)

Normocytic

• Renal anaemia / chronic disease -> UEC, iron studies
• Haemolysis (LDH, retic, haptoglobin, Coombs)
• Bone marrow failure -> reticulocytes
• acute blood loss

Macrocytic

• Vit B12 / folate
• TFT / liver function
• MDS (film)
• Medications

Question 2

DRILL = BMT hx

Answer 2

• Indication
  
  • Type (allo / auto)
    ○ Allo -> Donor, ?well-matched
  • Pre-Tx regimen
    ○ Conditioning -> myeloablative or not
  • Pre-engraftment period
    ○ Febrile neutropaenia
    ○ Cytopaenias
  • Post-engraftment
    ○ GVHD
  • Immunosuppression regimen + Prophylaxis
  • Complications of immunosuppression
    ○ Infertility
    ○ Infection
    ○ Further malignancy -> surveillance
  • Prognosis
    ○ Relapse
  • Monitoring (Chimerism)

Question 3

What are the classic manifestations of acute GVHD?

What is the difference between presentation of acute and chronic?

Answer 3

Multi-organ syndrome of inflammation / fibrosis

Acute = <100 days ( T cell)

- Skin / Eyes 
- Liver
- Mouth / GIT

Chronic = >100 days ( B cell)
- Above but more lung

Question 4

How would you manage this person’s GHVD?

Answer 4

Prevention and Treatment of GVHD centres around immunosuppression of donor cells
-> This is complex, as it requires careful balancing of treating the GVHD without overly affecting the graft vs tumour effect

Mx Plan:
- Site-specific Rx (if mild)
○ Skin -> emollients, topical steroids
○ Mouth -> dental hygiene, saliva
○ Eyes -> artificial tears
○ GIT -> evaluate diarrhoea for c.diff, CMV, scope
- Systemic immunosuppression (if severe)
○ Prednisolone at lowest possible dose
○ Calcineurin inhibitor for steroid-sparing
- Prevention of infection
○ Risk -> relates to organ damage AND immunosuppression
○ Appropriate Px as per guidelines / expert advice
○ Vaccines

Question 5

What would your long term care of this person post BMT be?

Answer 5

1. Monitoring for relapse
   
   2. Monitoring for second cancers
   3. Infection Prophylaxis
      a. PJP (in all) -> Bactrim
      b. CMV (depending on status) -> Valganciclovir
      c. HSV/VSV (IgG positive) -> Valaciclovir
      d. Fungal -> not for everyone. Fluconazole or Posa/Vori
      e. Encapsulated organisms (if GVHD) -> Penicillin
   4. GVHD Rx
   5. Maintaining health
      a. CV risk
      b. Mental health
      c. Vaccines

Question 6

What are the high risk thrombophilias?

Answer 6

• Protein C / S deficiency
• Antithrombin III deficiency
• Prothrombin gene mutation / FVL homoz
• Compound heterozygote PT / FVL

Question 7

Indications for warfarin over NOAC?

Answer 7

• mechanical heart valve
• mitral stenosis / valvular AF
• severe renal impairment
• APLS
• very high BMI with uncertain drug exposure

Question 8

How can myeloma affect the kidney?

Answer 8

• Cast nephropathy -> The urine dipstick is typically negative for protein as it is primarily Bence-Jones proteinuria (monoclonal light chains in urine)
• AL amyloidosis / light chain deposition disease -> markedly positive dipstick for protein, with nephrotic syndrome

Question 9

HAS - BLED score?

Answer 9

Hypertension 
Abnormal renal and/or hepatic function 
Stroke 
Bleeding tendency/predisposition 
Labile INR on warfarin 
Elderly (age >65 years) 
Drugs (aspirin or NSAIDs) and/or alcohol

Question 10

What determines if myeloma is ‘symptomatic’?

Answer 10

CRAB

• hypercalcaemia
• renal failure
• anaemia
• bone lesions (on low dose CT)

Question 11

How to interpret SPEP / SFLC etc?

Answer 11

SPEP will show if there is a band/peak to indicate a monoclonal paraprotein

Serum FLC:

• measures kappa and lambda light immunoglobulin chains that are unbound to heavy chains in the serum.
• Normal kappa/lambda FLC ratio is 0.26 to 1.65.
• Abnormal FLC ratios are seen in light chain myeloma

Urine PEP -> will pick up Bence-Jones protein

Question 12

How are you going to balance the risk of bleeding vs thrombosis in this person? Will you anticoagulate them? (think of Rhonda)

Answer 12

This is a complex issue and there are many considerations, particularly what the patients preferences are.

I would consider =

What is the indication for anticoagulation?
- is it very strong e.g. mechanical valve? Or is it a fairly low CHADSVasc score with no previous stroke?

What is the bleeding risk in this person?

• quantify using HASBLED score, but look at co-morbidities such as renal/liver failure, HTN
• are they having frequent falls?
• have they bled before?

Are there modifiable risk factors?

• HTN
• other meds e.g. NSAIDs
• falls risk

If overall, the benefit outweighs the risk then I would recommend anticoagulation.

Which agent?

• NOACs generally have lower bleeding rates and less monitoring
• However warfarin is reversible. Dabigatran is now too

Question 13

If someone bled on anticoagulation, how would you approach restarting it?

Answer 13

This depends on the type of bleed and location.

Often, the patient’s anticoagulant can be resumed within a period of approximately two weeks following resolution of the bleed. However if it was a spontaneous intracerebral bleed this could >4 weeks and should be discussed with the neurosurgical team