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Hemoglobin
international journal for hemoglobin research
Volume 36, 2012 - Issue 3
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Short Communication

Nondeletional Hb Queens Park [α32(B13)Met→Lys]/Hb H (β4) Disease

Suravee SroymoraBlood Disease Diagnostic Center, Hematology Division, Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Sumalee JindadamrongwechBlood Disease Diagnostic Center, Hematology Division, Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence[email protected]
,
Punnee ButthepBlood Disease Diagnostic Center, Hematology Division, Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
&
Suporn ChuncharuneeDepartment of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
Pages 293-298 | Received 10 Oct 2011, Accepted 22 Dec 2011, Published online: 02 Mar 2012
 
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Abstract

A rare nondeletional α-thalassemia-2 (α-thal-2) allele was identified in a Thai boy with Hb H (β4) disease. The proband has α-thal-1 (– –SEA type) together with a non productive Hb Queens Park (HBA1:c.98T>A) [α32(B13)Met→Lys] α1-globin variant. No abnormal hemoglobin (Hb) fraction was detected by high performance liquid chromatography (HPLC). The clinical effect of this mutation in the proband was comparable to that of deletional α-thal-2 present in Hb H disease.

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