Hemoglobin Elect

2 tubes EDTA whole blood

LAV-T/2

Tuesday and Thursday

The major hemoglobin in the erythrocytes of the normal adult is HbA with small amounts of HbA2 and HbF possible.

Most hemoglobin variants cause no discernible clinical symptoms, so are of interest primarily to research scientists. Variants are clinically important when their presence leads to sickling disorders, thalassemia syndromes, The combinations of Hb S-S, Hb S-D-Los Angeles, and Hb S-O-Arab lead to serious sickling disorders.2 Several variants including Hb H, EFort Worth and Lepore cause a thalassemic blood picture.2 The two variant hemoglobins of greatest importance in the U.S., in terms of frequency and pathology are HbS and HbC.2 Sickle cell anemia (HbSS) is a cruel and lethal disease. It first manifests itself at about 5-6 months of age. The clinical course presents agonizing episodes of pain and temperature elevations with anemia, listlessness, lethargy, and infarct in virtually all organs of the body. The individual with homozygous HbCC suffers mild hemolytic anemia which is attributed to the precipitation or crystallization of HbC within the erythrocytes. Cases of HbSC disease are characterized by hemolytic anemia that is milder than sickle-cell anemia.
The thalassemias are a group of hemoglobin disorders characterized by hypochromia and microcytosis due to the diminished synthesis of one globin chain (the a or b) while synthesis of the other chain proceeds normally.  This unbalanced synthesis results in unstable globin chains. These precipitate within the red cell, forming inclusion bodies that shorten the life span of the cell. In a-thalassemias the a-chains are diminished or absent, and in the b-thalassemia the b-chains are affected. Another quantitative disorder of hemoglobin synthesis, hereditary persistent fetal hemoglobin (HPFH), represents a genetic failure of the mechanisms that turn off gamma chain synthesis at about four months after birth which results in a continued high percentage of HbF. It is a more benign condition than the true thalassemias and persons homozygous for HPFH have normal development, are asymptomatic and have no anemia. The most common hemoglobin abnormalities:

Sickle Cell Trait - This is a heterozygous state showing HbA and HbS and a normal amount of HbA2 on cellulose acetate. Results on citrate agar show hemoglobins in the HbAand HbS migratory positions (zones).
Sickle Cell Anemia - This is a homozygous state showing almost exclusively HbS, although a small amount of HbF may also be present.
Sickle-C Disease - This is a heterozygous state demonstrating HbS and HbC.
Sickle Cell-Thalassemia Disease - This condition shows HbA, HbF, HbS, and HbA2.
In Sickle Cell b°-Thalassemia HbAis absent.
In Sickle Cell b+-Thalassemia HbA is present in reduced quantities.
Thalassemia-C Disease - This condition shows HbA, HbF and HbC.
C Disease - This is a homozygous state showing almost exclusively HbC.
Thalassemia Major - This condition shows HbF, HbAand HbA2.