Imbalanced globin chain synthesis in cultured erythroid progenitor cells from thalassemic bone marrow and peripheral blood.

The plasma clot culture system was utilized to support the growth of erythroid-committed stem cells from the bone marrow of five patients with homozygous -thalassemia and the peripheral blood of one patient doubly heterozygous for j9#{176}-thalassemia and Hb Lepore. Addition of erythropoietin to the cultures resulted in abundant colony growth (76-185 CFU-E and 3.5-11 BFU-E/6 x i0 bone marrow cells). Eight BFU-E I 1 0’ cells were assayed from the peripheral blood of the /1#{176}-thalassemia/Hb Lepore patient. Globin chain synthetic ratios of CFU-Eand BFUE-derived colonies were determined by CMC column chromatography after labeling of the cultures with 3H-leucine for 24 hr prior to peak growth. Absent or decreased fi-globin chain synthesis was observed in all cases; $/cx