Uncommon association of hypertriglyceridemia and autoimmune haemolytic anaemia in an infant with transfusion dependent thalassemia

Abstract Background Hypertriglyceridemia thalassemia syndrome (HTS) is not a well understood entity, and is mainly identified in the Indian subcontinent. We present a unique case of this syndrome along with severe autoimmune haemolysis that presented with life-threatening anaemia. Observations An infant with transfusion dependent thalassemia (TDT) presented with suspected mismatched blood transfusion leading to severe haemolysis. Severe haemolysis, lipemic serum were initial findings. Detailed evaluation revealed IgG, C3d positive mixed autoimmune haemolytic anaemia (AIHA), severe hypertriglyceridemia that improved with steroids and least mismatched packed red cell transfusion. Conclusion Children with thalassemia presenting with excessive hemolysis may have mismatched transfusion reaction, alloimmunization or an autoimmune hemolysis. Baseline extended blood grouping, detailed immune hemolytic work-up aids in prompt diagnosis. HTS on a background of autoimmune hemolysis is rare and needs urgent medical support.