A Rare Hemoglobinopathy Presenting as Chronic Hyperbilirubinemia

Authors

  • Lenaa Sakthiyavathy Manivannan Postgraduate, Department of Pediatrics, Sri Manakula Vinayagar Medical College and Hospital, Madagadipet, Pondicherry, India
  • Kanimozhi Thandapani Assistant Professor, Department of Pediatrics, Sri Manakula Vinayagar Medical College and Hospital, Madagadipet, Pondicherry, India
  • Arulkumaran Arunagirinathan Professor and Head, Department of Pediatrics, Sri Manakula Vinayagar Medical College and Hospital, Madagadipet, Pondicherry, India

Keywords:

Sickle beta thalassemia, Hb electrophoresis, HbS, HbF, HbA

Abstract

Sickle beta thalassemia represents the double heterozygous state of HbS and beta-thalassemia genes. Clinical manifestation varies from those indistinguishable from homozygous sickle cell anemia to completely asymptomatic ones. This disorder is diagnosed by increased levels of HbS, HbF, mildly increased HbA2 and varying levels of HbA on Hemoglobin electrophoresis. We report a 13 yrs. old girl who presented to us with jaundice for one year with hepatosplenomegaly and no history of blood transfusions or sickle cell crisis. Hb electrophoresis clinched the diagnosis of sickle beta thalassemia.

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Published

20-08-2021

How to Cite

1.
Manivannan LS, Thandapani K, Arunagirinathan A. A Rare Hemoglobinopathy Presenting as Chronic Hyperbilirubinemia. JK Science [Internet]. 2021 Aug. 20 [cited 2024 Nov. 21];23(3):162-4. Available from: https://journal.jkscience.org/index.php/JK-Science/article/view/79

Issue

Section

CASE REPORTS