História clínica

3-year old girl with fever of unknown origin for 10 days, pancytopenia and significant hepatosplenomegaly. She was a normally developed child of Asian ethnicity with no significant past medical and/or family history. Four days later she required admission to pediatric intensive care unit for septic shock and multi organ failure. On admission her laboratory values were as follows: WBC 2.7 K/uL, RBC 2.9 M/uL, HGB 7.7 G/dL, HCT 22.1, PLT 24 K/uL. She had BUN of 41 mg/dL, elevated liver function tests AST and ALT of 763 and 147 IU/L respectively and Billirubin total 11.2 and direct 8.3 mg/dl. In addition her work up was remarkable for markedly elevated ferritin of >40000 ng/ml, LDH of 5744 IU/L and markedly elevated triglycerides of 324 mg/dl. A bone marrow was performed.