Differences between individuals with hemoglobins Istanbul and Saint-Etienne (alpha 2 beta 2 92F8 His replaced by Gln).
Acta Haematol, 1979;61(5):295-7.
PMID: 111458
Impact factor: 3.068
Abstract
The unstable hemoglobins Istanbul and Saint-Etienne have the same amino acid substitution (alpha 2 beta 2 92F8 His leads to Gln). Despite this, there are some clinical and hematological differences between the individual with Hb Istanbul and the one with Hb Saint-Etienne. These are: (1) the clinical course of the patient with Hb Istanbul before splenectomy was more severe when compared with that of the individual with Hb Saint-Etienne; (2) although Hb F was found within normal range in the individual with Hb Istanbul, a high level of Hb F (19%) was observed in the patient with Hb Saint-Etienne. The alkali-resistant Hb found in the former was of adult type and in the latter case it was of fetal type. The possible explanations for these differences are discussed.
MeSH terms
Adult; Amino Acids; Child; Fetal Hemoglobin; Hemoglobins, Abnormal; Humans; Male; Mutation; Splenectomy
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