Hemoglobin J—As a Cause of Congenital Hemolytic Anemia

Abstract

Hemoglobin-J is a rare hemoglobin variant known to be clinically silent most of the times, only to be detected accidentally. Herein, the authors report a case of Hemoglobin-J manifesting as unstable hemoglobin detected during evaluation of hemolytic anemia in an 8 month-old-infant. Cation Exchange-High Performance Liquid Chromatography(CE-HPLC) was used to identify this variant after Hb electrophoresis was reported to be normal.

Hemoglobin J in a patient with severe anemia, a case report from Nepal

“…Hence, HPLC and hemoglobin electrophoresis is suggested after a time of 12 weeks from the last transfusion. Though rare in its presentation, hemolytic anemia associated with Hb J is reported in a few instances, especially when oxidative damage precipitated an unstable form in HbJ as demonstrated by the presence of Heinz bodies in peripheral smear [ 14 , 15 ].…”