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Phase 2 N=2 Supportive Care

Cyclophosphamide in Treating Young Patients With Severe Autoimmune Enteropathy

Diarrhea · Gastrointestinal Complications · Unspecified Childhood Solid Tumor, Protocol Specific

Enrolled (actual)
2
Serious AEs
0.0%
Results posted
Apr 2019
Primary outcome: Primary: Number of Participants With Treatment-free Remission at 1 Year After Study Completion — 2 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
filgrastim (Biological); cyclophosphamide (Drug)
Age
Pediatric, Adult · 1+ yrs
Sex
All
Sponsor
Johns Hopkins University
Primary completion
Feb 2009

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Participants With Treatment-free Remission at 1 Year After Study Completion
2
PRIMARY
Number of Participants Experiencing Intervention-related Adverse Events, as Defined by CTCAE at 1 Month

Summary

RATIONALE: Cyclophosphamide may help control the symptoms of autoimmune enteropathy . PURPOSE: This phase II trial is studying how well cyclophosphamide works in treating young patients with severe autoimmune enteropathy.

Eligibility Criteria

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)
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00258180). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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