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Flashcards in Heme disorders Deck (17):
1

Sideroblastic anemia can be from what? (its basically difficult to make RBCs)

Genetic Defect in heme making

Acquired:
Alcohol Abuse
Isonizide

2

Lab for sideroblsatic anemia?

Microcytic
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

3

Tx sideroblastic anemia?

Vitamin B6 (pyridoxime)
Treat underlying cause

4

Alpha thalaseemia

Defect in alpha globin (you have 4 alleles for it)
African and asian

5

Beta thalasemia

Seen in mediterranean

6

Alpha thalassemia minima?

1 mutated allele. Minimal disease and asymptomatic

7

Alpha thal minor?

2 mutated alleles, Mild microcytic anemia

8

HbH disease?

3 mutated alpha alleles, so you have all beta global

HEMOGLOBIN H: Shift to left and binds to oxygen too tight

chronic hemolysis

9

Hemoglobin Bart's?

Baby can only make gaba global

Incompatible with life. HYDROPS fettles b/c can't let go of oxygen

10

Beta globin has 2 alleles

Beta thal minor?

Decreased Beta global

Increased HbA2 which is alpha globin and delta globin

11

Beta thal minor is what?

NO Beta

Have HbA2
HbF (fetal

12

Smear seen with thalllasemia?

TARGET CELLS

13

Iron overload happens b/c lots of transfusion with thallesemia. Tx for that?

chelate iron

Deferoxamine

14

Dx of thalllasemia?

Do iron studies first

Then hemoglobin electrophoresis

15

What does hydroxyurea do?

More hemoglobin F in sickle cell (no need for beta globin)

16

Kid with sickle cell gets what?

Vaccines to encapsulated (SHiN)

Prophylaxis with penicillin until age 5 for pneumococcal

17

complication in 10% of sideroblastic anemia?

Acute leukemia