A Pilot Study of the Combination of Retinoic Acid and Interferon-Alpha2a for the Treatment of Lymphoproliferative Disorders in Children With Immunodeficiency Syndromes

Study start date: June 1, 1995

DISEASE CHARACTERISTICS: Congenital or acquired immunodeficiency (including HIV-1 infection) with a lymphoproliferative disorder (LPD) of any of the following types: Severe and/or progressive lymphadenopathy with hypergammaglobulinemia. Diffuse infiltrative lymphocytosis syndrome. Clinically symptomatic pulmonary lymphoid hyperplasia/lymphocytic interstitial pneumonitis. Polyclonal B-cell LPD. No patients with malignant lymphoma. No active opportunistic infection requiring acute intervention at entry. THERAPY: Biologic Therapy: At least 30 days since immunomodulating agents or biological response modifiers, e.g.: Interleukin-2, Interferons, Growth hormone, Insulin-like growth factor 1. Requirement waived for intravenous immunoglobulins for hypogammaglobulinemia. Concurrent post-transplant immunosuppressants allowed. Doses stable for at least 4 weeks prior to entry. Chemotherapy: At least 30 days since chemotherapy. Endocrine Therapy: Concurrent corticosteroids allowed only for lymphocytic interstitial pneumonitis or an autoimmune process. Doses stable for more than 4 weeks prior to entry. Radiotherapy: At least 30 days since radiotherapy. Surgery: Not specified. Antiretroviral therapy (in patients with HIV infection): Approved anti-HIV medication required. Initiated at least 8 weeks prior to entry. Continued throughout protocol treatment. Prophylaxis for Pneumocystis carinii pneumonia and/or Mycobacterium avium-intracellulare allowed. Maintenance antifungal or antiviral therapy allowed. PATIENT CHARACTERISTICS: Age: Under 18. Performance status: Not specified. One or more of the following laboratory findings (within 4 weeks of starting retinoic acid and interferon-alpha , and which have not resolved within 2 weeks of starting): Creatinine greater than 2 times the upper limit of normal; Liver transaminases greater than 5 times the upper limit of normal (children with chronically elevated liver enzymes with a proven etiology can be enrolled, but will not be evaluable for liver toxicity); or Bilirubin greater than 3 times the upper limit of normal. Patients receiving treatment for an acute infection must have completed therapy at least 14 days prior to starting therapy with retinoic acid and interferon-alpha. OTHER: Able to swallow capsules. No requirement for drugs suspected of causing pseudotumor cerebri for which alternatives cannot be substituted, e.g.: Tetracycline, Nalidixic acid, Nitrofurantoin, Phenytoin, Lithium, Amiodarone, Vitamin A (except as a multivitamin supplement component). No critical or clinically unstable illness. No pregnant or nursing women. Effective contraception encouraged in fertile patients. Parent or legal guardian available to give informed consent and deemed sufficiently reliable to return for followup visits. No critically ill or critically unstable children.