Pancytopaenia Flashcards
define pancytopaenia
deficiency of blood cells of all lineages (but generally excludes lymphocytes)
is pancytopaenia a diagnosis
no
does pancytopaenia always mean there is bone marrow failure
no
what are the 2 main processes behind pancytopaenia
reduced production
increased destruction
what can cause reduced production in pancytopaenia
inherited bone marrow failure syndromes
acquired bone marrow failure
example of inherited bone marrow failure syndrome
Fanconi’s anaemia
what are the 3 features of inherited bone marrow failure syndrome
abnormal haemopoeisis
cancer predisposition
congenital anomalies
list causes of primary acquired bone marrow failure
Idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia
what is idiopathic aplastic anaemia
autoimmune condition attaking HSCs resulting in deficiency of mature blood cells
features of myelodysplastic syndromes
dysplasia - disordered development
ineffective haemopoeisis
how can acute leukaemia result in acquired bone marrow failure
Leukaemia stem cells proliferate to form abnormal ‘blasts’ which do not differentiate into mature blood cells meaning there is a deficiency in these cells
list causes of secondary acquired bone marrow failure
Drugs B12/folate deficiency Infection Infiltration HIV, storage diseases
which drugs can cause bone marrow failure
chemotherapy
chloramphenicol
alcohol
B12 and folate deficiencies result in abnormal nuclear maturation in ALL lineages, true or false
TRUE
what results in increased destruction as a cause of pancytopaenia
hypersplenism
what is meant by increased splenic pool
there is an increased splenic red cell mass and platelet pol
slow transit time for red cells
an enlarged spleen means hypersplenism, true or false
false, may not always correlate
what are clinical features of pancytopaenia
anaemia: fatigue, pallor, SOB, CVS compromise
neutropaenia: infections
thrombocytopaenia: bleeding and bruising
Investigations for pancytopaenia
History and examination FBC Blood film (U+E, LFT, serology, virology, B12, folate) Bone marrow/Trephine biopsy (cytogenetics)
Aplastic anaemia causes bone marrow HYPO/HYPERcelllularity
HYPOcellular
Do the following cause HYPO/HYPERcellular bone marrow:
Myelodysplastic syndromes
B12 and folate deficiencies
Hypersplenism
HYPERcellular
how can you treat pancytopaenia supportively
red cell transfusion
platelet transfusion
(neutrophil transfusion)
prophylactic antibiotics/antifungals
in a patient with neutropaenic fever, should you wait for the microbiology results before giving antibiotics
no
give them ASAP
How do you treat pancytopaenia specifically
identify and treat the cause eg drugs - stop them malignancy - give chemo replace B12 and folate etc
