Hemoglobinopathies And Polycythemia Flashcards
Alpha like globin
Chromosome 16
B like globin
Chromosome 11
Predominant hemoglobin in adults
Hgb A
Causes by impaired production of polypeptide chains
Thalassemia
Severe hypochromic, microcytic anemia, hepatomegaly
Major beta thalassemia
Probably the most common single gene disorder in humans
Alpha thalassemia
Complete absence of alpha chains
Barts hydrops fetalis
3 out of 4 alpha is missing
Hgb H
Most common hemoglobinopathy
Sickle cell trait AS
Found mostly among blacks
HbSS
Sickle cell disease
Positive for sickling test
Sickling disorder
Reduced plasma volume
Relative PV
An increase in HCT and RBC
Not total red cell mass
Relative PV
BM is erythroid hyperplasia
Absolute PV
Commonly used tracer for erythrocyte volume
51Cr