ALPHA-THALASSEMIA AND BETA-THALASSEMIA IN A TURKISH FAMILY
AMERICAN JOURNAL OF HEMATOLOGY, sa.1, ss.1-15, 1977 (SCI-Expanded)
- Yayın Türü: Makale / Tam Makale
- Basım Tarihi: 1977
- Doi Numarası: 10.1002/ajh.2830020102
- Dergi Adı: AMERICAN JOURNAL OF HEMATOLOGY
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED)
- Sayfa Sayıları: ss.1-15
- Hacettepe Üniversitesi Adresli: Evet
Özet
A Turkish family is described in which 3 children have a clinical picture similar to that of thalassemia major, with typical red cell morphology and indices, and with about 10% Hb Bart''s but without measurable amounts of Hb H. Hematological evaluation of 6 members of this family that included in vitro Hb synthesis suggests that .beta.- (or .delta..beta.-) thalassemia, .beta.-silent thalassemia, and mild and severe .alpha.-thalassemia genes are present in different combinations. .beta./.alpha. chain ratios in patients with more than 1 type of thalassemia should be evaluated in relation to values obtained for several relatives even though some of the thalassemia determinants may be silent in the parents.