PROLONGED RESOLUTION OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS FOLLOWING MYELOABLATIVE CHEMOTHERAPY AND SUBSEQUENT AUTOLOGOUS PERIPHERAL-BLOOD STEM-CELL TRANSPLANTATION

A 30-month-old boy with hemophagocytic lymphohistiocytosis (HLH) recei ved an autologous peripheral blood stem cell transplant (PBSCT) follow ing high-dose chemotherapy. He presented with hemophagocytic syndrome (HPS) at 6 months of age, but relapsed despite the repeated administra tion of prednisolone, VP-16, cyclosporin A (CsA), and other cytotoxic agents, PBSC were obtained using combination chemotherapy with etoposi de (VP16, 450 mg/m(2)), doxorubicin (70 mg/m(2)), vincristine (2 mg/m( 2)) and cyclophosphamide (CY, 1200 mg/m(2)), 2.7 x 10(5)/kg CFU-GM PBS C were transplanted after similar high-dose VP16 preconditioning used for allogeneic BMT for HLH, The boy continues to remain in complete re mission 30 months after PBSCT while receiving low-dose PSL/CsA therapy , High-dose chemotherapy followed by PBSCT may be an optional therapeu tic approach for patients with HLH.