Anemia part 3 Flashcards
Intrinsic inherited hemolytic anemias: qualitative defects
sickle cell disease and HbC disease
what does the peripheral blood smear of a single RBC look like in hereditary spherocytosis
lack central palor
hereditary spherocytosis is a mutation in what? which produces what
RBC membrane skeleton protein gene, ankyrin
- anchor
what happens to hereditary spherocytosis when RBC pass through spleen
become spherocytes
hereditary spherocytosis deficiency is what
spectrin - membrane protien
how does one diagnose hereditary spherocytosis and are the results
osmotic fragility test
- microcytosis
- increase MCHC
hereditary spherocytosis treatment
folic acid supplements
- splenectomy
Glucose-6-phosphate dehydrogenase deficiency has a defective what
red cell enzyme
Glucose-6-phosphate dehydrogenase deficiency may protect from what disease
falciparum malaria
Glucose-6-phosphate do to the RBC
gives antioxidant protection
what happens to hemoglobin when there is oxidant stress without Glucose-6-phosphate dehydrogenase? what forms?
rbc denatured- forms Heinz body
what does Glucose-6-phosphate dehydrogenase make form NAD
NADPH
what is the role of NADPH
detoxify oxygen free radicals (hydrogen peroxide)
where are variants of Glucose-6-phosphate dehydrogenase deficiency located and severity
Mediterranean= severe
middle eastern
what are 3 precipitating factors for Glucose-6-phosphate dehydrogenase deficiency
infections
oxidant drugs ( sulfonamides, -quine, antimalarials)
fresh fava beans
is Glucose-6-phosphate dehydrogenase deficiency a constant occurrence in the body
no, acute attacks
what happens in autoimmune hemolytic anemias
autoantibodies attach to RBC
how is autoimmune hemolytic anemias detected
combs test