ALPHA-THALASSEMIA AND BETA-THALASSEMIA IN A TURKISH FAMILY

ALTAY C., SAY B., YETGIN S., HUISMAN T.

AMERICAN JOURNAL OF HEMATOLOGY, no.1, pp.1-15, 1977 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Publication Date: 1977
  • Doi Number: 10.1002/ajh.2830020102
  • Journal Name: AMERICAN JOURNAL OF HEMATOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED)
  • Page Numbers: pp.1-15
  • Hacettepe University Affiliated: Yes

Abstract

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.