Cyclophosphamide in Treating Young Patients With Severe Autoimmune Enteropathy

You may be eligible if

• DISEASE CHARACTERISTICS:
• Diagnosis of severe autoimmune enteropathy
• * Condition is resistant to conventional therapy
• Histologic evidence of severe villous atrophy with intense lymphocytic infiltrate of the lamina propria by small intestinal biopsy within the past 3 months
• Disease failed to respond after ≥ 2 months of corticosteroid therapy at a dose of ≥ 0.5 mg/kg/day or ≥ 40 mg/day for patients \> 20 kg AND 1 of the following therapies:
• * Cyclosporine resulting in ≥ 1 whole blood level of \> 200 ng/mL
• * Tacrolimus resulting in ≥ 1 whole blood level of 5 ng/mL
• At least 50% estimated caloric needs provided by parenteral nutrition
• History of intractable diarrhea, defined as frequent watery stools for \> 3 months that does not respond to dietary restriction
• No celiac disease, defined by a history of positive antiendomysial antibody or tissue transglutaminase antibody
• No primary immunodeficiency or x-linked autoimmunity-allergy dysregulation
• PATIENT CHARACTERISTICS:
• Performance status
• Lansky 60-100%
• Life expectancy
• Not specified
• Hematopoietic
• Not specified
• Hepatic
• Not specified
• Renal
• Not specified
• Cardiovascular
• Ejection fraction ≥ 40% OR shortening fraction ≥ 20%
• Pulmonary
• FVC or FEV\_1 ≥ 50% of predicted (for patients \> 8 years of age)
• No clinically abnormal pulmonary function or abnormal pulse oximetry (for patients ≤ 8 years of age)
• Other
• Not pregnant
• Negative pregnancy test
• Fertile patients must use effective contraception during and for at least 9 months after completion of study treatment
• No known chromosomal abnormality
• PRIOR CONCURRENT THERAPY:
• Biologic therapy
• No immunizations for at least 6 months after completion of study treatment
• Endocrine therapy
• See Disease Characteristics
• At least 5 days since prior corticosteroids
• No concurrent dexamethasone as an anti-emetic
• Other
• At least 5 days since other prior immunosuppressive medications (e.g., tacrolimus or cyclosporine)