B3.047 Hypoproliferative Anemia Flashcards
what is the best way to differentiate between inadequate and ineffective production from BM?
look at a bone marrow aspirate
full- ineffective
empty- inadequate
what are some potential causes of inadequate BM production?
bone marrow failure
bone marrow infiltration/ replacement (myelophthisis)
nutritional deficiency
anemia of chronic disease
what are some reasons for bone marrow failure? which is most common?
aplastic anemia
pure red cell aplasia
iatrogenic - most common, many immunosuppressive meds
what are some ways that ineffective production in the BM manifests?
cells collect in BM and don’t leave
OR
rapid destruction within the BM
what is aplastic anemia?
myeloid stem cell disorder
marrow hypoplasia and pancytopenia
aplastic anemia epidemiology
incidence - 2-5 cases / 1,000,000 per year
clinical features of aplastic anemia
anemia
leukopenia
thrombocytopenia
no splenomegaly
why is aplastic anemia a misnomer?
hypoplastic, not completely aplastic
all myeloid cell lines, not just RBCs
pathogenesis of aplastic anemia
primary stem cell abnormality
OR
immune mediated stem cell destruction
immunological factors causing aplastic anemia
idiopathic
viral infections
environmental factors causing aplastic anemia
drugs/chemicals
whole body radiation
viral infections
genetic factors causing aplastic anemia
Fanconi
telomerase defects
acquired stem cell defects
potential for acquisition of additional mutations and transformation into AML
aplastic anemia blood path
pancytopenia
normochromic, normocytic RBCs, no poikilocytes
reticulocytopenia
aplastic anemia BM path
hypocellular
decreases hematopoiesis
increased marrow fat
aplastic anemia treatment
supportive (transfusions)
immunosuppression (see if it works to differentiate between immunologic or clonal)
androgens
hematopoietic stem cell transplant
aplastic anemia prognosis
1 year without treatment
75% 5 year survival with transplant
50-70% response to immunosuppression
what is pure red cell aplasia
disorder or erythroid progenitor cell causing decreased erythropoiesis
epidemiology PRCA
very rare