BONE MARROW TRANSPLANTATION IN THALASSEMIA
Samar Emad EI-Din EI-Mehallawy;
Abstract
The Thalassemias are a heterogeneous group of genetic
disorders of hemoglobin synthesis, all of which result from a reduced rate of production of one or more of the globin chains of hemoglobin.
They are divided into the a, 13, 813 or y813 thalassemias, according to which globin chain is produced in reduced amount. In some thalassemias, no globin chain 1s synthesized at all, and hence they are called a0 or 13o thalassemias, whereas, in others, some globin chain 1s produced but at reduced rate; these are designated a+ or 13+ thalassemias.
Thalassemia 1s considered the most common genetic disorder worldwide. It occurs in a particularly high frequency in a broad belt extending from the Mediterranean basin through the Middle East, Indian subcontinent, Burma, and Southeast Asia.
Most of the a thalassemia syndromes result from gene deletions. The a globin genes appear to be predisposed to deletions because of tandemly duplicated sequences in the a
gene cluster. In contrast, most of the 13 thalassemia
syndromes result from one or more nucleotide substitutions or deletions in genes that are otherwise intact.
disorders of hemoglobin synthesis, all of which result from a reduced rate of production of one or more of the globin chains of hemoglobin.
They are divided into the a, 13, 813 or y813 thalassemias, according to which globin chain is produced in reduced amount. In some thalassemias, no globin chain 1s synthesized at all, and hence they are called a0 or 13o thalassemias, whereas, in others, some globin chain 1s produced but at reduced rate; these are designated a+ or 13+ thalassemias.
Thalassemia 1s considered the most common genetic disorder worldwide. It occurs in a particularly high frequency in a broad belt extending from the Mediterranean basin through the Middle East, Indian subcontinent, Burma, and Southeast Asia.
Most of the a thalassemia syndromes result from gene deletions. The a globin genes appear to be predisposed to deletions because of tandemly duplicated sequences in the a
gene cluster. In contrast, most of the 13 thalassemia
syndromes result from one or more nucleotide substitutions or deletions in genes that are otherwise intact.
Other data
| Title | BONE MARROW TRANSPLANTATION IN THALASSEMIA | Other Titles | زرع نخاع العظام فى انيميا البحر المتوسط | Authors | Samar Emad EI-Din EI-Mehallawy | Issue Date | 2000 |
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