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

Hemoglobin J is defined by a faster movement towards anode when compared with the normal hemoglobin A. Though a pathologically distinct entity from the normal HbA, it remains clinically silent due to little physiological difference as exemplified by a similar oxygen binding capacity between the two. Though cases of symptomatic HbJ have been reported, it is uncommon. Hence, further explanations should be sought in such cases.

Our case report exemplifies the presence of an alpha thalassemia trait along with HbJ in a symptomatic case of anemia from rural Nepal.

CE-HPLC complemented by electrophoresis, is the method of choice for characterizing various hemoglobin variants including Hb J. Hb J presents as elevated P3 peak on HPLC while thalassemia is detected by the presence of eluted proteins at the retention time between 0 and 1 minutes. P3 peak up to 6% is considered normal, values 6%-12% indicates suboptimal specimen and values greater than 15% indicates Hb J.

Variants of hemoglobin including HbJ variant is detected using HPLC technique. Mostly clinically silent, if HbJ is associated with anemia, search for a concomitant cause should be sought one of them being alpha thalassemia when iron deficiency has been ruled out by a serum iron profile.