Pancytopaenia + Plasma dyscrasias

Question 1

Lifespan of a RBC

Answer 1

120 days

Question 2

Lifespan of a neutrophil

Answer 2

7-8hrs

Question 3

Lifespan of platelets

Answer 3

7-10 days

Question 4

Pathophysiology of Fanconi’s anaemia (inherited bone marrow failure syndrome)

Answer 4

Unable to correct inter-strand cross-links (DNA damage)

Question 5

Clinical features of Fanconi’s anaemia

Answer 5

Short stature
Skin pigment abnormalities
Radial ray abnormalities
Hypogenitilia
Endocrinopathies
GI defects
Cardiovascular
Renal
Haematological

Question 6

Myelodysplasia may progress into …

Answer 6

Acute myeloid leukaemia

Question 7

Myelodysplasia - pancytopaenia?

Answer 7

Possibly, but most patients will have an isolated anaemia

Question 8

B12/folate deficiency causes secondary bone marrow failure

Answer 8

Associated with a hypercellular marrow

Question 9

Drug-induced secondary bone marrow failure

Answer 9

Chemotherapy, alcohol, azathioprine, methotrexate chloramphenicol

Question 10

Causes of hypersplenism

Answer 10

Splenic Congestion
- Portal Hypertension

Systemic diseases
- Rheumatoid Arthritis

Haematological diseases
- Splenic lymphoma

Question 11

Summary of pancytopaenia

Answer 11

Question 12

Clinical features of anaemia

Answer 12

Fatigue
Shortness of breath
Cardiovascular compromise

Question 13

Clinical features of neutropaenia

Answer 13

Infections

Gram negative sepsis particularly life-threatening in the short-term.
Gram positive bacteria can also cause damage but not death within hours, if untreated.

Invasive fungal infections

Question 14

Clinical features of thrombocytopaenia

Answer 14

Bleeding
- Purpura
- Petechiae
- ‘Wet’ bleeds including visceral bleeds

Question 15

Marrow cellularity in pancytopaenia - hypo/hypercellular ?

Answer 15

Hypocellular in aplastic anaemia

Hypercellular in
- Myelodysplastic syndromes
- B12/folate deficiency
- Hypersplenism

Question 16

Treatment for neutropaenic fever?

Answer 16

Treat immediately with antibiotics

Don’t wait for lab results

Question 17

Amyloid diagnosis

Answer 17

Organ biopsy - Congo red stain, “Apple-green” birefringence under polarised light

Question 18

How to prevent tumour lysis syndrome?

Answer 18

Aggressive hydration + allopurinol/rasburicase

Question 19

Medication used to correct hypercalcaemia

Answer 19

Bisphosphonates e.g. pamidronate

Inhibits osteoclasts (therefore limiting bone destruction)

Question 20

Treatment for myeloma

Answer 20

Combination novel agent chemotherapy
- Corticosteroids: dexamethasone
- ‘Novel agents’ like bortezomib and lenalidomide
- Monoclonal antibodies: e.g., daratumumab
- Alkylating agents: cyclophosphamide, melphalan used less these days

High-dose chemotherapy/autologous stem cell transplant in fit patients

(Use paraprotein levels to monitor response)

Question 21

What additional treatment is used to make very high-dose chemotherapy safer?

Answer 21

AHSCT (autologous haematopoietic stem cell transplant)

Question 22

Amyloidosis treatment

Answer 22

Similar to myeloma.

High-dose chemotherapy

Question 23

Clinical features of Waldenstrom’s macroglobulinaemia

Answer 23

• Hyperviscosity syndrome
• Fatigue, visual disturbance, confusion, coma
• Bleeding
• Cardiac failure
• B symptoms; night sweats, weight loss

Question 24

Treatment for Waldenstrom’s macroglobulinaemia

Answer 24

• Chemotherapy
• Plasmapheresis (removes paraprotein from the circulation)

Question 25

Treatment of hyperviscosity

Answer 25

- Remove patient plasma rich in IgM paraprotein - Replace with donor plasma - Viscosity reduced

Question 26

Protein associated with Waldenstrom's macroglobulinaemia

Answer 26

IgM paraprotein

Question 27

Unique feature of IgM antibody?

Answer 27

Pentameric Therefore, increased risk of hyperviscosity