TY - JOUR
T1 - Sickle and thalassemic erythroid progenitor cells are different from normal
AU - Alter, Blanche P.
AU - He, Liya
AU - Acosta, Robert
AU - Knobloch, Mary Ellen
AU - Thomson, Joann C.
AU - Giardina, Patricia
AU - Weinberg, Rona S.
PY - 1992
Y1 - 1992
N2 - Blood erythroid progenitors (BFU-E) from patients with sickle and thalassemic syndromes were compared with those from normal individuals. The day of maximal colony formation in methyl cellulose was slightly later in the cultures from the patients with hemoglobinopathies than in the normal cultures. The number of colonies/100,000 mononuclear cells was similar in all cultures on day 13, but was higher in the hemoglobinopathy cultures on the day of maximal growth. The number of BFU-E/mL of blood was significantly higher than normal at all times in both sickle cell anemia and thalassemia. The proportional synthesis of γ globin was twice normal in all sickle cultures, and 4 times normal in those from β+-thalassemia. Hemin and interleukin-3 increased the numbers of erythroid colonies in all cultures, but did not consistently alter the globin synthesis patterns. Each progenitor population has a unique pattern in terms of time course, number of BFU-E, and level of γ globin synthesis. These features indicate distinct types of BFU-E, or differences in accessory cells, or both, which distinguish blood-borne erythropoiesis in normals and those with hemoglobinopathies.
AB - Blood erythroid progenitors (BFU-E) from patients with sickle and thalassemic syndromes were compared with those from normal individuals. The day of maximal colony formation in methyl cellulose was slightly later in the cultures from the patients with hemoglobinopathies than in the normal cultures. The number of colonies/100,000 mononuclear cells was similar in all cultures on day 13, but was higher in the hemoglobinopathy cultures on the day of maximal growth. The number of BFU-E/mL of blood was significantly higher than normal at all times in both sickle cell anemia and thalassemia. The proportional synthesis of γ globin was twice normal in all sickle cultures, and 4 times normal in those from β+-thalassemia. Hemin and interleukin-3 increased the numbers of erythroid colonies in all cultures, but did not consistently alter the globin synthesis patterns. Each progenitor population has a unique pattern in terms of time course, number of BFU-E, and level of γ globin synthesis. These features indicate distinct types of BFU-E, or differences in accessory cells, or both, which distinguish blood-borne erythropoiesis in normals and those with hemoglobinopathies.
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U2 - 10.3109/03630269208993114
DO - 10.3109/03630269208993114
M3 - Article
C2 - 1487417
AN - SCOPUS:0027098047
VL - 16
SP - 447
EP - 467
JO - Hemoglobin
JF - Hemoglobin
SN - 0363-0269
IS - 6
ER -