Pancytopenia Flashcards
pancytopenia
deficiency of blood cells of all lineages
red cell lifespan
120 days
neutrophil lifespan
7-8 hours
platelets lifespan
7-10 days
causes of pancytopenia
reduced production or increased destruction
reduced production causes
bone marrow failure
acquired is most common - primary or secondary
or inherited
characterisitics of inherited marrow failure syndromes
cancer pre dis
impaired haemopoiesis
congenital anomalies
arise due to defects in dna repair/ ribosomes/ telomeres
example of inherited marrow failure syndrome
very rare
fanconis anaemia
clinical features of fanconis anaemia
Short stature
Skin pigment abnormalities
Radial ray abnormalities
Hypogenitilia
Endocrinopathies
GI defects
Cardiovascular
Renal
Haematological
skeletal and skin changes children with fanconis anaemia may have
cafe au lait spots
skeletal abnoramlities
haematological abnormalities of inherited marrow failure syndromes
Median age: 7 years
Unable to correct inter-strand cross-links (DNA damage)
Macrocytosis followed by thrombocytopenia, then neutropenia
Bone marrow failure (aplasia) risk: 84% by 20 years
Leukaemia risk: 52% by 40 years
examples oof intrinsic problem with haemopoietic stem cell
idiopathich aplastic anaemia; autoimmune attack against cell
myelodysplastic syndromes
acute leukaemia
myelodysplastic syndromes characterisitcs
cloncal haemopoetic stem cell disorder
hypercellular marrow
increased apoptosis of progenitor and mature cells
do all patients with myelodysplastic syndromes have pancytopenia
no , isolated anaemia is most common
what might myelodysplastic syndromes lead to
acute myeloid leukaemia
examples of secondary bone marrow failurw
drug induced - alcohol, azathioprine, methotrexate, chloramphenicol , chemo
b12 folate def
infiltrative
viral- hiv
increased destruction causes
hypersplenism
hypercellular marrow as trying to compensate
Increased destruction that exceeds bone marrow capacity, usually associated with significantly enlarged spleen
causes of hypersplenism
Splenic Congestion
Portal Hypertension
Systemic diseases
Rheumatoid Arthritis
Haematological diseases
Splenic lymphoma
clincial features of pancytopenia
. Anaemia
Fatigue
Shortness of breath
Cardiovascular compromise
Neutropenia
Infections
Severity
Duration
. Thrombocytopenia
Bleeding
Purpura
Petechiae
‘Wet’ bleeds including visceral bleeds
what can be fatal in those with neutropenia
gram neg bacteria infections
which conditions show hypercellularity
Myelodysplastic syndromes
B12/folate deficiency
Hypersplenism
which condition shows hypocelullarity
aplastic anaemia
supportive treatment
Supportive
Red cell transfusions
Platelet transfusions
Neutrophil transfusions not routine
Antibiotics prophylaxis/treatment
antibacterials*
antifungals
specific treatment for primary bone marrow disorder
Malignancy – consider treatment (chemotherapy)
Congenital – consider allogeneic stem cell transplantation
Idiopathic Aplastic Anaemia – Immunosuppression
MDS – depends on risk of leukaemic transformation, erythropoiesis-stimulating agents in low risk, demethylating agents or chemotherapy in higher risk
specific treatment in secondary bone marrow disorder
Drug reaction – STOP
Viral – eg treat HIV
Replace B12/folate
hypersplenism treatment
treat cause if possible
consider splenectomy
neutropenic fever treatment
treat promptly based on local unit antibiotic policy without waiting for microbiology results
