Background: HbH disease is a nonfatal form of alpha thalassemia syndrome. The alpha thalassemia syndromes are a group of hereditary anemias of varying clinical severity and characterized by reduced or absent production of one or more of the globin chains of hemoglobin molecule. HbH disease is common in Indian Subcontinent but not all the cases detected are reported in literature. Here we report a case of HbH disease in a three year of female patient who presented with history of mild fever and mild distension of abdomen for 15 days.Complete blood count showed HB4.4 gm%, TLC – 10.6 X 103/uL, platelet count was 358 X 103/uL. 8 nucleated RBCs were seen per 100 WBCs. She was given iron therapy for one month but showed mild response only. So a hemoglobin electrophoresis was done.A peripheral blood film was prepared which showed presence of microcytes, few macrocytes, teat drop cells, fragmented cells and polychromatophils and NRBCs. Reticulocyte preparation showed 7% reticulocytes with occasional HbH like inclusions. Hb electrophoresis was done on capillary electrophoresis ( Mini cap, Sebia ) which revealed a HbH band at zone 15. Conclusion: HbH disease is a gentic disease with 25% chance of acquiring the disease with each pregnancy when the partner carries a mutated alpha globin gene. Hence prenatal genetic counselling of family members is necessary. A high index of suspicion is required for a prompt diagnosis and management.

Keywords: HbH Disease; Alpha Thalessemia; Hb Electrophoresis.