Aplastic anaemia or bone marrow failure
Description
- Pancytopenia + hypocellular marrow, no infiltrate, no dysplasia, no fibrosis
- A hypocellular marrow in a pancytopenic patient = aplastic anaemia (acquired or inherited) OR hypoplastic MDS. Separating these is the whole diagnostic task
Categories
- Acquired AA - ~80% idiopathic, immune-mediated
- Inherited bone marrow failure - Fanconi, dyskeratosis congenita, Shwachman-Diamond, Diamond-Blackfan
- Single-lineage failure - pure red cell aplasia, congenital amegakaryocytic thrombocytopenia
- Congenital dyserythropoietic anaemias - anaemia + ineffective erythropoiesis + secondary iron overload from inc gut absorption
Severity - Camitta criteria
- Severe: marrow cellularity <25%, PLUS 2 of 3:
- Neutrophils <0.5
- Platelets <20
- Reticulocytes <20 x10^9/L
- Very severe: as above with neutrophils <0.2
- Non-severe: everything else
- Severity drives urgency and eligibility for definitive therapy, nothing else
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