Pancytopenia

Question 1

What is pancytopenia?

Answer 1

deficiency of blood cells of all lineages

Question 2

What are the two causes of pancytopenia?

Answer 2

reduced production

increased destruction

Question 3

What causes reduced production?

Answer 3

bone marrow failure

Question 4

What is an inherited cause of bone marrow failure?

Answer 4

Fanconis anaemia

Question 5

How does Fanconis anaemia present?

Answer 5

median age - 7 short stature
skin pigment abnormalties
radical ray abnormalities
hypogenitialia 
cafe au lait spots

Question 6

What is seen on blood film in Fanconis anaemia?

Answer 6

Macrocytosis followed by thrombocytopenia then neutropenia - high risk of leukaemia

Question 7

What are primary aquired cause of bone marrow failure?

Answer 7

idiopathic aplastic anaemia - autoimmune attack against HSC stopping formation of granulocytes, erythrocytes and platelets
myelodysplastic syndrome
acute leukaemia

Question 8

What are secondary aquired cause of bone marrow failure?

Answer 8

drug induced - chemo, drugs, alcohol, chloramphenicol
B12/folate deficiency
non haemopoietic malignant infiltration
storage disease

Question 9

What causes increased destruction to cells?

Answer 9

Hypersplenism - spleen detects, retains and eliminates unwanted, forgein or damaged material

Question 10

What can cause hypersplenism?

Answer 10

splenic congestion: portal hypertension and congestive cardiac failure
systemic diseases: rheumatoid arthritis
haematological diseases: splenic lymphoma

Question 11

What are the general features of pancytopenia?

Answer 11

anaemia
neutropenia
thrombocytopenia

Question 12

What treatment is given for anaplastic anaemia?

Answer 12

immunosurpression