Pancytopenia Flashcards
define pancytopenia
deficiency of blood cells of ALL lineages
but generally excludes lymphocytes
what is important to remember about pancytopenia
NOT a diagnosis
life span of mature RBCs, platelets, neutrophils
RBC- 120 days
Neutrophil- 7-8 hours
Platelet- 7-10 days
what are the 2 mechanisms through which pancytopenia can happen
reduced production (marrow failure) increased destruction (hypersplenism)
causes of reduced production
bone marrow failure
- inherited
- acquired (primary- aplastic, secondary drugs)
what is the genetic fault in inherited marrow failure syndromes
defects in DNA repair and ribosomes
example of an inherited bone marrow failure
fanconi’s anaemia
features of fanconi’s anaemia
short stature cafe au lait spots hypogenitalia GI, cardio, renal defects aplastic anaemia skeletal abnormalities e.g. missing thumbs
haematological abnormalities in fanconi’s anaemia
unable to correct DNA damage
macrocytosis followed by thrombocytopenia, then neutropenia
Can progress to bone marrow failure + leukaemia
mean age onset of fanconi’s anaemia
7 years old
what is a primary acquired bone marrow failure
e.g. aplastic anaemia
an intrinsic marrow problem
usually a stem cell defect
causes of primary bone marrow failure
aplastic anaemia
myelodysplastic syndromes
acute leukaemia
what is aplastic anaemia
autoimmune attack against haemopoietic stem cells
what is seen in myelodysplastic syndromes
dysplasia
hypercellular marrow – marrow producing more cells but they die more prematurely so lots of immature cells
increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)
what can myelodysplastic syndrome evolve into
acute myeloid dysplasia
causes of secondary bone marrow failure
drug induced – chemo, chloramphenicol, alcohol
B12/folate deficiency– hypercellular
Lymphoma
HIV
causes of increased RBC destruction
hypersplenism
what is hypersplenism
increased destruction that exceeds bone marrow capacity generate new cells
what leads to hypersplenism
splenomegaly
causes of splenomegaly
splenic congestion
- portal hypertension, HF, portal/splenic vein occlusion
systemic disease
- RA
haematological diseases
- splenic lymphoma, haemolytic anaemia,
blood findings in pancytopenia
anaemia, neutropenia, thrombocytopenia
what is seen in neutropenia
repeated infections
what is seen in thrombocytopenia
bleeding
- purpura
- petechia
tests to find the cause of pancytopenia
History + family history clinical findings FBC B12/Folate/LFTs/virology depending on history bone marrow examination
specialised test for fanconi’s anaemia
chromosome fragility
what marrow cellularity is seen in aplastic anaemia
hypocellular - marrow failure therefore no cells being produced
when is hyper cellular marrow seen
myelodysplastic syndromes
B12/folate deficiency
hypersplenism
bone marrow packed full of immature cells
types of treatment for pancytopenia
supportive
specific
supportive treatment
red cell transfusion
platelet transfusion
antibiotic treatment + prophylactic use
specific treatment for primary bone marrow failure
malignancy
- chemotherapy
congenital
- bone marrow transplantation
aplastic anaemia
- immunosuppression
specific treatment for secondary bone marrow failure
drug reaction
- stop the drug
viral
- treat cause
replace B12/Folate if deficient
splenectomy
what causes hyper cellular bone marrow
B12/Folate deficiency
myeloproliferative disorders
hypersplenism
infections that can cause pancytopenia
HIV
EBV
Paravirus
Hepatitis
drugs that can cause pancytopenia
cytotoxic: azathioprine, methotrexate
antibiotics: chloramphenicol
diuretics: thiazides
hyperthyroid: carbimazole
anti-pychotics: clozapine
anti-epileptic: phenytoin
