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Hemoglobin D (Hb D Punjab/ Los Angeles and Hb D Iran) and Co-Inheritance with Alpha- and Beta- Thalassemia in southern Iran

AUTHORS

M Zakerinia 1 , * , M Ayatollahi 2 , M Rastegar 2 , Sh Amanat 2 , AR Askarinejad 2 , S Amirghofran 2 , M Haghshenas 2

AUTHORS INFORMATION

1 Associate Professor of Internal Medicine, Hematology Research Center, Organ Transplant Research Center, School of Medicine, Shiraz University of Medical Sciences, transbon@sums.ac.ir, Fars, Iran

2 Organ Transplant Research Center, Nemazee Hospital, Stem Cell and Transgenic Technology Research Center, Shiraz University of Medical Sciences, Fars, Iran

How to Cite: Zakerinia M, Ayatollahi M, Rastegar M, Amanat S, Askarinejad A, et al. Hemoglobin D (Hb D Punjab/ Los Angeles and Hb D Iran) and Co-Inheritance with Alpha- and Beta- Thalassemia in southern Iran, Iran Red Crescent Med J. Online ahead of Print ; 13(7):493-498.

ARTICLE INFORMATION

Iranian Red Crescent Medical Journal: 13 (7); 493-498
Article Type: Research Article
Received: November 2, 2010
Accepted: February 10, 2011

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Abstract

Background: Hemoglobin-D (Hb D) is an uncommon structural hemoglobin variant, which is reported to be prevalent in north western India. There are only a few small series, of this entity in the literature. We report the largest single center experience on this entity from Iran.

Methods: Between November 2002 and December 2010 as a result of screening premaritally for beta-thalassemia in Shiraz, Fars Province, Southern Iran, column chromatography, Hb electrophoresis, solubility test, and/or high performance liquid chromatography (HPLC), direct sequencing and restriction analysis were used for hemoglobinopathies and structural Hb variants. The data of 220 subjects with Hb D variants are analyzed in this report.

Results: These comprised of 180 carries of Hb D; 92 cases of Hb D Punjab/Los Angeles (b121Glutamic acid®Glutamine) and 88 subjects with Hb D Iran
 (b 22 Glu®Gln), 3 homozygous cases for Hb D, 17 subjects with beta- thalassemia- Hb D, 12 with Hb D- alpha- thalassemia- 1, 3 homozygous Hb D- alpha thalassemia- 1 trait, one with Hb D Punjab - sickle cell anemia, and two with Hb D Iran/sickle cell anemia.

Conclusion: The carriers of Hb D and homozygous cases for Hb D were not anemic and had normal red blood cell morphology, as they are not usually detected. If Hb D was inherited in combination with thalassemia, the subjects had mild anemia and in some of them, the spleen was palpable (1-2 cm). Co-inheritance of alpha thalassemia and Hb D resulted in the slightly higher Hb level and lower Hb D level as compared to Hb D/ beta- thalassemia cases (Hb D 24-37% vs 57-88%). Co inheritance of Hb D and sickle cell results was moderate to severe hemolytic anemia.

Keywords

Hemoglobin (Hb) D Punjab Hb D Iran Hb D- beta- thalassemia (thal) Hb D-alpha- thal-1 trait Hb DSickle cell anemia

© 0, Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

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