EBV associated hemophagocytic lymphohistiocytosis with spontaneous regression.

INDIAN PEDIATRICS 1253 VOLUME 42DECEMBER 17, 2005 A 9-year-old boy, previously healthy, was referred to our center with 10 days of fever, weakness, epistaxis and purpura. Examination revealed a febrile child who had marked pallor; petechiae over limbs and trunk; 1-1.5 cm multiple sub-mental lymph nodes; 3 cm hepatomegaly and 5 cm splenomegaly below costal margins respectively. Investigations (Table I) showed pancytopenia with lymphocytosis; elevated ALT and AST; repeated blood cultures were sterile. Bone marrow aspiration (BMA) revealed dyserythropoiesis and several histiocytes, some of which were exhibiting erythrophagocytosis suggestive of hemophagocytic lymphohistiocytosis (HLH). Further investigations revealed corroborative evidence of HLH in the form of elevated serum LDH, triglyceride and ferritin levels. Hepatitis virus serology was suggestive of an old infection with hepatitis B virus. Serology for Ebstein Barr Virus (EBV) was suggestive of recent infection. Serum immunoglobulins revealed mild reduction in IgA and IgM levels. Thus final diagnosis was EBV associated HLH. Patient showed remarkable clinical improve-ment with supportive care including intra-venous antibiotics, platelet and blood trans-fusions. Within a week, he was asymptomatic and follow-up showed gradual improvement with normalization of biochemical abnormalities and BMA at 6 weeks. At 15 months of follow-up, he remains asymptomatic.