Title of article
A Compound Heterozygous of Hb E and Beta Thalassemia Independent of Transfusion: A Rare Case Report
Author/Authors
Khairwa ، Anju Departments of Pathology - University College of Medical Sciences GTB hospital , Kotru ، Mrinalini Departments of Pathology - University College of Medical Sciences GTB hospital , Dewan ، Pooja Departments of Pathology - University College of Medical Sciences GTB hospital
From page
315
To page
319
Abstract
Hemoglobinopathy is now a worldwide problem. Mainly described in Southeast Asian countries. A compound heterozygous form of hemoglobin (Hb) E β thalassemia was reported to be 50% of all severe forms of thalassemia. Clinical presentation of HbE/ b thalassemia is reported to be moderate to severe anemia. The children usually present at the age range of 7 months to 6.9 years. The clinical severity is affected by genetic factors, including mutation in a chain (β thalassemia), alpha-chain (a thalassemia), amount of adult Hb, and co-inheritance of another hemoglobinopathy. Here, we present a rare case of compound heterozygous of HbE/ β thalassemia), who survived without transfusion and complication until eight years of age. Peripheral blood smears and complete blood count showed microcytic hypochromic anemia. High-performance chromatography showed a compound heterozygous of HbE/ β thalassemia.
Keywords
Compound , Hemoglobin E , Heterozygous , Transfusion
Journal title
Iranian Journal of Pediatric Hematology and Oncology
Journal title
Iranian Journal of Pediatric Hematology and Oncology
Record number
2765009
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