Hemoglobin d (hb d punjab los angeles and hb d Iran)‎ and co-inheritance with alpha- and beta- thalassemia in southern Iran

Abstract EN

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 premarital 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 (β 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.