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Postnatal changes in the quantities of globin chains and hemoglobin types in two babies with Hb H disease
Author(s) -
McKie K. M.,
Gu L.H.,
Gu Y.C.,
Huisman T. H. J.
Publication year - 1993
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.2830420117
Subject(s) - hemoglobin , fetal hemoglobin , globin , anemia , fetus , medicine , biology , chemistry , genetics , pregnancy
We have studied two babies with Hb H disease from birth to about six months of age and analyzed the changes in the relative quantities of the five globin chains (α, β, G γ, A γ) and the four hemoglobins (Hb F, Hb A, Hb Bart's, Hb H) using different high performance liquid chromatography procedures. The types of Hb H disease were —(SEA)/‐α (3.7 kb) and —(Fil)/‐α (3.7 kb); the larger—(Fil) deletion includes the functional 2‐globin gene, explaining the higher chain level in the baby with the —(SEA)/‐α (3.7 kb) type. The functional hemoglobin level at birth (Hb A + Hb F) was 11 to 12 g/dl with 3 to 4 g/dl Hb Bart's (γ4). Only 5% of the “fast‐moving” hemoglobin was Hb H (β 4 ). The level of Hb F at birth was low (less than 50% of the total Hb A + Hb F). After birth, the α and γ chain production decreases rapidly resulting in a severe anemia (total functional hemoglobin ∼7 g/dl) at 30 to 60 days postnatally, improving gradually to 8.5–9.5 g/dl at age of three months. The preferential formation of Hb A over Hb F at birth, and presumably prenatally, has the advantage that the level of the highly unstable Hb H is kept low; it also results in low levels of Hb F impairing the oxygen transfer capability of the fetal blood. © 1993 Wiley‐Liss, Inc.