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α‐Thalassemia and β‐thalassemia in a turkish family
Author(s) -
Altay C.,
Say B.,
Yetgin S.,
Huisman Titus H. J.
Publication year - 1977
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830020102
Subject(s) - thalassemia , turkish , medicine , hemoglobin , hemoglobinopathy , pediatrics , genetics , hemolytic anemia , biology , philosophy , linguistics
A Turkish family is described in which three 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 six members of this family that included in vitro hemoglobin synthesis suggests that β‐ (or δβ‐) thalassemia, β‐silent thalassemia, and mild and severe α‐thalassemia genes are present in different combinations. The data indicate that β/α chain ratios in patients with more than one type of thalassemia should be evaluated in relationship to values obtained for several relatives even though some of the thalassemia determinants may be silent in the parents.

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