Hb J Calabria is a fast moving hemoglobin variant which was found in an Italian family by Vecchio et al. (1), and in a French family by Blouquit et al.who studied its functional properties (2). The original family described by Vecchio et al. in which both Hb J Calabria and βthal-assemia were present has been reexamined and is the subject of the present study. Hematological and clinical features of the carriers are described. The heterozygous carriers of Hb J Calabria showed only mild variable sub-clinical anemia and levels of the abnormal hemoglobin ranging from about 33 to 42% The Hb J Calabria/βthal-assemia double heterozygote showed a moderate chronic hemolytic anemia with alterations of the RBC indices and morphology in addition to splenomegaly. The relationship between structural abnormality, functional properties and clinical expression of Hb J Calabria is discussed. © 1979 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.
Studies on a family with HB j calabria (α2 β2 64 (e8 GLY → ASP) / Marinucci, M.; Mavilio, F.; Fontanarosa, P. P.; Tentori, L.; Brancati, C.. - In: HEMOGLOBIN. - ISSN 0363-0269. - 3:5(1979), pp. 327-340. [10.3109/03630267908997538]
Studies on a family with HB j calabria (α2 β2 64 (e8 GLY → ASP)
Mavilio F.;
1979
Abstract
Hb J Calabria is a fast moving hemoglobin variant which was found in an Italian family by Vecchio et al. (1), and in a French family by Blouquit et al.who studied its functional properties (2). The original family described by Vecchio et al. in which both Hb J Calabria and βthal-assemia were present has been reexamined and is the subject of the present study. Hematological and clinical features of the carriers are described. The heterozygous carriers of Hb J Calabria showed only mild variable sub-clinical anemia and levels of the abnormal hemoglobin ranging from about 33 to 42% The Hb J Calabria/βthal-assemia double heterozygote showed a moderate chronic hemolytic anemia with alterations of the RBC indices and morphology in addition to splenomegaly. The relationship between structural abnormality, functional properties and clinical expression of Hb J Calabria is discussed. © 1979 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.
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