Haematology

Question 1

Multiple myeloma

Answer 1

• A malignancy of plasma cells (fully differential B Cells)
• Always effects bone marrow
• Destructive bone changes leading to pain & pathological fractures
• Excess plasma cells in the bone marrow

Question 2

Burkitts Lymphoma

Answer 2

• Highly aggressive B Cell Lymphoma
• African endemic
• Young patients
• “starry sky” appearance of plasma cells

Question 3

Philadelphia chromosome

Answer 3

• t(9:22)

- CML

Question 4

B Cell Lymphoma

Answer 4

• One of Kappa and Lambda

Question 5

CML

Answer 5

• Markers - CD5, CD23, CD19, CD20
• Only treat symptomatic disease
• Expression of 1 type of light chain (Kappa or Lambda)

Question 6

Renal Disease in MM

Answer 6

# Cast nephropathy
- Tubular disease-direct damage to PCT with reduced re- absorption of LC with co-ppt with Tamm-Horsfall protein in distal tubules - most common

Question 7

MM

Answer 7

• Light Chain deposition disease: also known as monoclonal immunoglobulin deposition disease, glomerular disease - less common
• High albumin in urine

Question 8

CML Treatment

Answer 8

1st line treatment is tyrosine kinase inhibitors;
- Imantinib
- Nilotinib; increased rates of molecular response and reduced rates of progression to blast crisis when compared to imantinib, but increased CV risk
• Avoid in patients with significant CV risk
- Dasatinib; similar responses to imantanib but SE profile worse
• Avoid in elderly & those with COPD
# Allogenic stem cell transplant – consider for patients with tyrosine kinase resistant disease or those with advanced disease eg accelerated phase or blast crisis
# Interferon alpha; previously a standard of care, now used occasionally after failure on TKIs or during pregnancy
# Hydroxyurea and bisulphan; old agents, now rarely used for CML

Question 9

Howell-Jolly Bodies

Answer 9

• Small basophilic collections in the RBC.
• These are in fact small clusters of DNA, which are remnant from when the RBC expelled its nuclus in the bone marrow.
• In the normal person the spleen “pits” these out.

Question 10

Intravascular haemolysis

Answer 10

• Urine haemosiderin - specific

- High LDH, High Bilirubin, low haptagloboin

Question 11

Cryoprecipitate

Answer 11

• Fibrinogen 150–250 mg with a half-life of 100–150 hours
• Factor VIII 80-150 U with a half-life of 12 hours
• von Willebrand factor 100-150 U with a half-life of 24 hours
• Factor XIII 50-75 U with a half-life of 150–300 hours.