Flashcards in Heme disorders Deck (17):
Sideroblastic anemia can be from what? (its basically difficult to make RBCs)
Genetic Defect in heme making
Lab for sideroblsatic anemia?
RDW is high
Ringed sideroblast in bone marrow (you just see small microcytic in periphery)
Sideroblasts are normal, but when they are ringed it is abnormal
Tx sideroblastic anemia?
Vitamin B6 (pyridoxime)
Treat underlying cause
Defect in alpha globin (you have 4 alleles for it)
African and asian
Seen in mediterranean
Alpha thalassemia minima?
1 mutated allele. Minimal disease and asymptomatic
Alpha thal minor?
2 mutated alleles, Mild microcytic anemia
3 mutated alpha alleles, so you have all beta global
HEMOGLOBIN H: Shift to left and binds to oxygen too tight
Baby can only make gaba global
Incompatible with life. HYDROPS fettles b/c can't let go of oxygen
Beta globin has 2 alleles
Beta thal minor?
Decreased Beta global
Increased HbA2 which is alpha globin and delta globin
Beta thal minor is what?
Smear seen with thalllasemia?
Iron overload happens b/c lots of transfusion with thallesemia. Tx for that?
Dx of thalllasemia?
Do iron studies first
Then hemoglobin electrophoresis
What does hydroxyurea do?
More hemoglobin F in sickle cell (no need for beta globin)
Kid with sickle cell gets what?
Vaccines to encapsulated (SHiN)
Prophylaxis with penicillin until age 5 for pneumococcal