Beta O-thalassemia intermedia
G Cividalli, H Kerem, E Ezeckiel and EA Rachmilewitz
Three patients with a relatively mild form of beta O-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 O-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 one case was smaller than in other
patients with "classical" Cooley anemia. It is concluded that the severity
of the clinical course in beta O-thalassemia does not correlate with the
imbalance in alpha verus gamma chain synthesis in peripheral blood and is
determined by the synthetic ratio in bone marrow cells, where the bulk of
hemoglobin synthesis takes place.
Volume 52,
Issue 2,
pp. 345-349,
08/01/1978
Copyright © 1978 by The American Society of Hematology
