Hemophagocytic lymphohistiocytosis following dengue hemorrhagic fever in Hb H/Hb Constant Spring patient.

摘要: Infection-associated hemophagocytic syndrome (IAHS), a secondary form of lymphohistiocytosis (HLH), has been found following several types infections and can be fatal. We report herein case IAHS dengue infection in 14-year-old patient with underlying α-thalassemia (non-deletional Hb H/Hb Constant Spring disease). He developed prolonged fever, thrombocytopenia, progressive splenomegaly. Further investigations indicated hyperferritinemia, increased reactive histiocytes activity the bone marrow. responded promptly to dexamethasone i.v. immune globulin. Physicians should aware this condition, especially countries where both hemorrhagic fever thalassemia are prevalent. The fatal outcome prevented prompt appropriate treatment.