pancytopenia Flashcards
what is pancytopenia
deficiency of all blood cells from all lineages (excluding lymphocytes)
in general terms, why does pancytopenia happen
decreased production of cells (eg bone marrow failure)
increased destruction of cells (eg hypersplenism)
causes of bone marrow failure
inherited diseases (eg faconi’s anaemia)
primary - aplastic anaemia, myelodysplastic syndromes, acute leukaemia
secondary - drug induced (chemo, chloramphenicol, alcohol,) B12 and folate, infiltrative from other malignancies, HIV
causes of hypersplenism
splenic congestion (portal hypertension, CCF) systemic diseases (eg rheumatoid arthritis) splenic lymphoma
features of faconi’s anaemia
7 years old risk of leukaemia short stature skin pigment abnormalities - café au lait spots endocrine abnormalities hypogenitilia GI, cardio, renal defects
what is aplastic anaemia
autoimmune condition against haemopoietic cells
what cytokines are involved in autoimmune aplastic anaemia
IFN-y
TNF-a
what haemopoietic cells are affected in autoimmune aplastic anaemia
long term and short term stem cells
multipotent progenitors
common myeloid precursor (eradicates RBCs, platelets and granulocytes)
what can trigger aplastic anaemia
parvovirus and hepatitis
presentation of pancytopenia
lack of blood cells anaemia (SOB, fatigue, palps, dizzy) neutropenia (recurrent infections) thrombocytopenia (bleeding - purpura, petechiae, visceral bleeds) also look for cause
when is bone marrow hypocellular in pancytopenia
aplastic anaemia
when is bone marrow hypercellular in pancytopenia
myelodysplastic syndromes (proliferation + apoptosis) B12/folate deficiency hypersplenism
supportive treatment in pancytopenia
red cell transfusions
platelet transfusions
prophylactic antibiotics, antifungals, antivirals
treatment for faconi’s anaemia
bone marrow transplant
treatment for autoimmune aplastic anaemia
immunosuppression
