Combination Chemotherapy Followed By Donor Stem Cell Transplant in Treating Patients With Hemophagocytic Lymphohistiocytosis
Nonneoplastic Condition
About this trial
This is an interventional treatment trial for Nonneoplastic Condition focused on measuring hemophagocytic lymphohistiocytosis
Eligibility Criteria
DISEASE CHARACTERISTICS: Newly diagnosed hemophagocytic lymphohistiocytosis (HLH) meeting 1 of the following criteria*: Diagnosis by molecular/genetic methods Diagnosis by meeting 5 out of 8 of the following criteria: Clinical criteria: Fever Splenomegaly Laboratory criteria: Cytopenias affecting ≥ 2 of 3 lineages in the peripheral blood, including the following: Hemoglobin < 9.0 g/dL (< 10.0 g/dL in infants < 4 weeks of age) Platelet count < 100,000/mm^3 Neutrophil count < 1,000/mm^3 Hypertriglyceridemia and/or hypofibrinogenemia: Fasting triglycerides ≥ 3.0 mmol/L (i.e., ≥ 265 mg/dL) Fibrinogen ≤ 1.5 g/L Histopathologic criteria: Hemophagocytosis in bone marrow, spleen, or lymph nodes No evidence of malignancy New diagnostic criteria: Low or absent natural killer (NK) cell activity Ferritin ≥ 500 mcg/L Soluble CD25 (i.e., soluble interleukin-2 receptor) ≥ 2,400 U/mL NOTE: *Patients who do not meet the diagnostic criteria for HLH but who have a strong clinical suspicion of HLH may be eligible at the discretion of the investigator Primary HLH (i.e., familial hemophagocytic lymphohistiocytosis [FLH]) OR secondary HLH (i.e., severe acquired form of HLH) Acceptable donor meeting 1 of the following criteria: HLA-identical related donor Matched unrelated donor Mismatched unrelated donor Familial haploidentical donor PATIENT CHARACTERISTICS: Not specified PRIOR CONCURRENT THERAPY: No prior cytotoxic treatment for HLH No prior cyclosporine treatment for HLH
Sites / Locations
- Birmingham Children's Hospital
- Institute of Child Health at University of Bristol
- Addenbrooke's Hospital
- Watford General Hospital
- Leeds Cancer Centre at St. James's University Hospital
- Royal Liverpool Children's Hospital, Alder Hey
- Great Ormond Street Hospital for Children
- Royal Manchester Children's Hospital
- Children's Hospital - Sheffield
- Southampton General Hospital
- Royal Aberdeen Children's Hospital
- Royal Hospital for Sick Children
- Royal Hospital for Sick Children