Hemoglobin Q disease

Hemoglobin Q is a relatively uncommon α - chain hemoglobin variant. heterozygotes of HbQ as well as double heterozygotes of HbQ and β- thalassemia are generally asymptomatic and the homozygous condition for this abnormal hemoglobin has not yet been reported. We report a 22 year old female with HbQ disease who came to us with sudden onset of anemia and splenohepatomegaly. Two of her other sibs who were also homozygous for HbQ were asymptomatic.