Pilot Study of Unrelated Donor Hematopoietic Stem Cell Transplantation in Patients With Life Threatening Hemophagocytic Disorders

PROTOCOL ENTRY CRITERIA: --Disease Characteristics-- Patients diagnosed with any of the following active but stable, or nonactive/quiescent, hemophagocytic disorders: * Hemophagocytic lymphohistiocytosis (HLH) * Fever greater than 38.5 degrees Celsius * Splenomegaly (greater than 3 cm below costal margin) * Hemophagocytosis in bone marrow or spleen or lymph nodes * Disease may be confirmed by positive family history * No evidence of malignancy * Hypertriglyceridemia and/or hypofibrinogenemia * Fasting triglycerides at least 2.0 mmol/L or at least 3 standard deviations above normal for age * Fibrinogen no greater than 1.5 g/L or no greater than 3 standard deviations above normal * Cytopenia (affecting at least 2 of 3 lineages in the peripheral blood) * Hemoglobin less than 9.0 g/L * Platelet count less than 100,000/mm3 X-linked lymphoproliferative disorder (XLP) Two or more maternally related males manifesting at least one of the following XLP phenotypes: * Fulminant infectious mononucleosis * Dysgammaglobulinemia * Malignant lymphoma/lymphoproliferative disorder * Aplastic anemia * Lymphoid granulomatosis/vasculitis OR * A maternally related male in an established XLP kindred who has strong genetic (RFLP) linkage to the XLP locus Chediak-Higashi syndrome Partial oculocutaneous albinism (hair, skin, eyes) Frequent bacterial infections Large peroxidase positive granules in leukocytes of peripheral blood or bone marrow Positive family history or parental consanguinity…