Hemoglobin E disorders in the north east India.

ABSTRACT

Clinical and hematological features of nine cases of Hemoglobin E variant were studied prospectively over three years. There were three cases of HbE-beta thalassemia and six cases of homozygous HbE disease. Two cases were asymptomatic. The commonest symptom was jaundice (unconjugated 2). Other symptoms were left hypochondrial pain, tiredness, syncope and failure to thrive. Splenomegaly and microcytosis was detected in all. Mild anaemia was present in majority (6/9), and the mean hemoglobin was 11.3 gm/dl. Reticulocyte count was normal in all and cholelithiasis was seen in one patient only. Clinical and hematological features were evaluated and compared to reported series. Splenomegaly was found to be more common.