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

INTRODUCTION

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.

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

DISCUSSION: 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.

CONCLUSION

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.