BETA-O-THALASSEMIA INTERMEDIA

  • 1 January 1978
    • journal article
    • research article
    • Vol. 52  (2) , 345-349
Abstract
Three patients with a relatively mild form of .beta.0-thalassemia, who did not require regular blood transfusions, are described. Globin synthesis was studied by gel filtration and urea-carboxymethylcellulose chromatography of stroma-free hemolysates prepared from peripheral blood and bone marrow cells labeled in vitro with 14C-leucine, .gamma./.alpha. Synthetic ratios in peripheral blood were in the same range as in patients with the severe clinical form of .beta.0-thalassemia, while .gamma./.alpha. synthetic ratios in bone marrow cells were higher than in that group of patients. The size of the free .alpha.-chain pool measured in 1 case was smaller than in other patients with classical Cooley anemia. The severity of the clinical course in .beta.0-thalassemia apparently does not correlate with the imbalance in .alpha. vs. .gamma. chain synthesis in peripheral blood and is determined by the synthetic ratio in bone marrow cells, where the bulk of Hb synthesis takes place.

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