Pancytopenia and bone marrow failure

Question 1

Define pancytopenia

Answer 1

a deficiency of all three cellular components of peripheral blood: red cells, white cells, and platelets.

Question 2

What are causes of pancytopenia?

Answer 2

1. Increase in one cell type with infiltration e.g. cancers
2. Failure of marrow production e.g. haematinic deficiency, aplastic anaemia, MDS
3. Peripheral destruction e.g. splenomegaly

Question 3

What are Sx of pancytopenia?

Answer 3

• Anaemia: fatigue, chest pain, SOB, pallor
  
  • Thrombocytopenia: epistaxis, bruising, bleeding
  • Neutropenia: increased frequency of infection

Invasion of peripheral blood and of organs/tissues cause
• hepatomegaly
• Splenomegaly
• Lymphadenopathy

Question 4

What Ix in pancytopenia?

Answer 4

FBC

Blood smear: can show reticulocytes

Haematinics

Bone marrow biopsy

flow cytometry etc

Question 5

What is agranulocytosis?

Answer 5

When WBCs with neutrophil, basophil, eosinophil granules are not made. Can be caused by carbimazole, procainamide, sulfonamides, clozapine

Question 6

What symptom should patients on drugs known to cause agranulocytosis report immediately?

Answer 6

Fever: can suggest agranulocytosis and patients need to be started on neutropaenic regimen.

Question 7

What Rx for marrow support in pancytopenia?

Answer 7

Red cell transfusion

Platelets. In patients who are immunosuppressed, they may need to be irradiated first to avoid GVHD. Give if platelets <10, haemorrhage e.g DIC, before invasive procedures

Neutropaenic regimen

Question 8

What is aplastic anaemia?

Answer 8

occurs when the bone marrow fails to produce enough blood cells, characterised by pancytopenia and a hypoplastic bone marrow

Question 9

What are features of aplastic anaemia?

Answer 9

• normochromic, normocytic anaemia
  
  • leukopenia, with lymphocytes relatively spared
  • thrombocytopenia

Can be presenting feature in acute leukaemia

Question 10

How is aplastic anaemia diagnosed?

Answer 10

Bone marrow biopsy: showing stem cells replaced by fat

also

FBC: pancytopenia

Question 11

What are causes of aplastic anaemia?

Answer 11

Congenital e.g. Fanconi syndrome

Drugs e.g. cytotoxics, chloramphenicol, sulphonamides, phenytoin

infections

Radiation

Question 12

What is Rx of aplastic anaemia?

Answer 12

Haematopoeitic stem cell transplant for young and fit people

Immunosuppression, steroids, chemo