Pancytopenia and Bone Marrow Failure Flashcards
What is pancytopenia?
This is a reduction in all major cell lines: red cells, white cells and platelets
What are causes of pancytopenia?
- Aplastic anaemia
- Infiltration
- Acute leukaemia
- Myelodysplasia
- Myeloma
- Lymphoma
- Disseminated TB
- Megaloblastic leukaemia
- Hypersplenism
What are primary causes of aplastic anaemia?
- Fanconi’s anaemia
- Idiopathic
What are secondary causes of aplastic anaemia?
- Chemicals
- Drugs - chemo, antibiotics
- Insecticides
- Ionising radiation
- Infections - HIV, EBV, TB, hepatitis
- Pregnancy
- Paroxysmal nocturnal haemoglobinuria
What is aplastic anaemia?
Defined as pancytopenia with hypocellularity (aplasia) of the bone marrow; there are no leukaemic, cancerous or other abnormal cells in the peripheral blood or bone marrow. It is usually an acquired condition but may rarely be inherited.
Caused a reduction in the number of pluripotential stem cells together with a fault in those remaining or an immune reaction against them so that they are unable to repopulate the bone marrow. Failure of only one cell line may also occur, resulting in isolated deficiencies such as the absence of red cell precursors in pure red cell aplasia.
How does aplastic anaemia present?
- Anaemia
- Infection
- Bleeding
What investigations would you perform if you suspected aplastic anaemia?
- Bloods - FBC
- Blood Film
- Bone marrow biopsy
What might you see on bone marrow biopsy in someone with aplastic anaemia?
A hypocellular or aplastic bone marrow with increased fat spaces
What would you see on FBC in someone with aplastic anaemia?
Pancytopenia
How would you manage someone with aplastic anaemia?
- Supportive management
- Bone marrow transplant
What is agranulocytosis?
Production of granulocytes has stopped (neutrophils, basophils, eosinophils), leaving the individual at risk of severe infection.
What can cause agranulocytosis?
Drugs - carbimazole, clozapine, sulfonamides
What is the normal lifespan of a platelet?
8 days
What is the average lifespan of neutrophils?
1-2 days
What are the most immediate problems in bone marrow failure?
- Thrombocytopenia
- Neutropenia