Abstract
We report a 15-month-old girl who developed hemophagocytic syndrome (HPS) 2 months before the diagnosis of acute lymphoblastic leukemia (ALL) with the 4 ; 11 translocation. She was admitted for persistent fever, pallor, hepatosplenomegaly, and severe pancytopenia and was diagnosed to have HPS. The clinical and laboratory findings were normalized by supportive care, including G-CSF administration. Two months after the onset of HPS, she was readmitted for an evaluation of fever, leukocytosis, and thrombocytopenia. The bone marrow specimen revealed a massive infiltration of lymphoblasts (L2 by FAB classification) without an increase in hemophagocytic cells. She was diagnosed by surface marker analysis as having B-precursor ALL and was successfully induced to complete remission. Because the blasts had the 4 ; 11 translocation and the MLL gene rearrangement, which predict poor prognosis of the disease, she received an allogeneic bone marrow transplantation from an HLA-identical sibling. At 2 years posttransplantation, she shows no recurrence of HPS or of ALL. The etiological relationship between HPS and ALL was discussed.
