pancytopenia Flashcards
what is pancytopenia
deficiency of blood cells of all lineages
causes of pancytopenia
reduced production
increased destruction
reduced production causes
bone marrow failure
- acquired (primary or secondary)
- inherited syndromes
inherited marrow failure syndrome example
fanconi anaemia
how do inherited syndromes causing marrow failure arise
due to defects in DNA repair/ ribosomes/telomeres
cause of primary acquired bone marrow failure
no obvious cause, usually stem cell defect
acquired primary bone marrow failure
idiopathic aplastic anaemia
myelodysplastic syndromes
acute leukaemia
what is idiopathic aplastic anaemia
autoreactive T cells target haemapoeisis
what is myelodysplastic syndromes
increased apoptosis of progenitor and mature cells, propensity for evolution into AML
secondary causes of marrow failure
drug induced- chemo, alcohol, azathioprine, methotrexate, chlorampenicol
B12/folate deficiency- nuclear maturation can affect all lineages
infiltrative- non-haemopoietic malignancy infiltration
viral- HIV
causes of increased destruction leading to pancytopenia
hypersplenism
- increased splenic pool
- increased destruction that exceeds bone marrow capacity
causes of hypersplenism
splenic congestion: portal hypertension
systemic diseases: rheumatoid arthritis
haematological diseases: splenic lymphoma
presentation of pancytopenia
anaemia
neutropenia
thrombocytopenia
investigations for pancytopenia
bloods- FBC, blood film
additional routine tests guided by history
bone marrow examination
treatment of pancytopenia
supportive: red cell, platelet transfusion, neutrophil transfusions not routine
ABx prophylaxis/treatment
