Pancytopenia

Question 1

Definition of pancytopenia

Answer 1

Reduction in all the major cell lines (red, white, platelets)

Question 2

Two broad causes of cytopenia

Answer 2

Reduced production or increased destruction

Question 3

Rare autosomal recessive disease causing bone marrow failure, usually by 20 years old

Answer 3

Fanconi anaemia

Question 4

Features of inherited marrow disorders (3)

Answer 4

Cancer predisposition
Congenital abnormalities
Impaired haemopoiesis

Question 5

Cause of aplastic anaemia

Answer 5

Autoimmune- triggered by infection (e.g. parvovirus), drugs, irradiation

Question 6

Difference between primary and secondary acquired bone marrow failure

Answer 6

Primary is intrinsic to the bone marrow

Question 7

Causes of secondary acquired bone marrow failure (3)

Answer 7

Drug-induced (e.g. chemo, chloramphenicol)
b12/folate deficiency
Malignant infiltration, lymphoma

Question 8

What is the underlying problem in myelodysplastic syndromes?

Answer 8

Increased apoptosis of progenitor and mature cells, causing ineffective haemopoiesis

Question 9

Definition of hypersplenism

Answer 9

Increased destruction of cells in the spleen exceeding compensatory capacity of the bone marrow, usually accompanied by splenomegaly

Question 10

Broadly what causes hypersplenism?

Answer 10

Anything that causes splenomegaly can cause hypersplenism

Question 11

Specific causes of hypersplenism (4)

Answer 11

Portal hypertension
Congestive cardiac failure
Rheumatoid arthritis
Haematological disease

Question 12

Haematological diseases causing hypersplenism (3)

Answer 12

Haemolytic anaemia
Leukaemia (especially CML)
Lymphoma

Question 13

Marrow cellularity in

a) aplastic anaemia
b) myelodysplastic syndromes
c) folate/B12 deficiency
d) hypersplenism

Answer 13

a) hypo
b) hyper
c) hyper
d) hyper

Question 14

Supportive management of marrow failure (3)

Answer 14

Red cell transfusions
Platelet transfusions
Prompt response to neutropenic fever (i.e. “blind” therapy)

Question 15

How is idiopathic aplastic anaemia treated?

Answer 15

Immunosuppression e.g. prednisolone, azathioprine, ciclosporin, MTX, cyclophosphamide

Question 16

causes of primary acquired pancytopenia (3)

Answer 16

aplastic anaemia
myelodysplastic syndrome
acute leukaemia

Question 17

Tests to establish the cause of a pancytopenia

Answer 17

FBC
Blood film
B12/folate
LFTs
Virology
Auto-antibody testing
Bone marrow biopsy