Defects of protoporphyrin synthesis Flashcards
What is the anemia when there is no protoporphyrin production
sideroblastic anemia
Anemia with very little protoporphyrin production?
Porphyria
What causes sideroblastic anemia
B6 responsive, lead poisoning, refractory
What is lead poisoning anemia treated with
EDTA chelation therapy`
Difference btwn ineffective erythropoiesis and normal
Normal: majority of cells move to differentiation>large quantity of mature cells
Ineffective: Majority of cells move to apoptosis. Only a few mature cells produced.
Traits of sideroblastic anemia
Ineffective erythropoiesis, ringed sideroblasts, increase in iron
What does a wrights stain show?
Pappenheimer bodies
What does a prussian blue stain show?
Siderotic granules
What are ringed sideroblasts?
Siderotic granules that are clustered around the nucleus instead of throughout
Serum iron inc or dec in sideroblastic anemia
inc
TIBC level for sideroblastic anemia
normal
Ferritin for sideroblastic anemia
Inc
% saturation for sideroblastic anemia
inc
Peripheral blood features sideroblastic anemia
Hypo micro
Pappenheimer bodies
basophilic stippling
What is seen in peripheral blood of refractory sideroblastic anemia
Variation in RBC chromia, anisochromia, dimorphic blood picture
What happens in congenital porphyria
Unstable protoporphyrin precursors deposit in various sites such as skin, fingernails, and teeth. Precipitates when exposed to sunlight
