Clinical Trial: Treatment and Natural History Study of Lymphomatoid Granulomatosis

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Interventional

Official Title: Treatment and Natural History Study of Lymphomatoid Granulomatosis

Brief Summary:

This study will evaluate the response and long-term effects of alpha-interferon in patients with lymphomatoid granulomatosis (LYG). The disease causes proliferation of destructive cells involving the lungs, skin, kidneys, and central nervous system.

Patients ages 12 and older who have LYG and who are not pregnant or breast feeding may be eligible for this study. Alpha interferon or chemotherapy, or both, will be used. Alpha interferon is a protein the body naturally produces. If patients have grade 3 disease, they will usually receive EPOCH-rituximab (EPOCH-R) chemotherapy (each letter representing a drug). If patients have grade 1 or 2 disease, the will usually receive alpha interferon. If patients have LYG after receiving alpha interferon and/or EPOCH-R, they may receive rituximab alone or with alpha interferon. Rituximab is an antibody, binding to a specific molecule (CD20) present on most B-cell lymphomas. Doses of several drugs in EPOCH-R may be increased if patients tolerated them in the previous cycle. If patients respond to EPOCH-R but still have low grade LYG, they may receive alpha interferon. Researchers will also try to obtain a biopsy of patients lesions, to help in understanding the disease.

Patients self-administer alpha interferon by injection under the skin three times weekly. They will visit the clinic every 2 to 12 weeks for follow-up. Patients will receive alpha interferon for 1 year after LYG goes away, depending on response. EPOCH-R has these drugs: rituximab by vein on Day 1; prednisone by mouth on Days 1 to 5; etoposide, doxorubicin, and vincristine as a continuous intravenous infusion on Days 1 to 5; and cyclophosphamide by intravenous injection over 1 hour on Day 5. Each cycle lasts 3 weeks: 5 days of chemotherapy and 16 days of no chemotherapy. Etoposide, doxorubicin, and vincristine are infused

Detailed Summary:

BACKGROUND:

  • Lymphomatoid granulomatosis (LYG) is an angiocentric destructive proliferation of lymphoid cells predominantly involving the lungs, skin, kidneys, and central nervous system.
  • It is divided into three grades, depending on the degree of necrosis and cellular atypia. The grades of disease are histologically-based and do not necessarily correlate with clinical outcome. However, like other EBV related LPD's, LYG can transform into an aggressive large B-cell lymphoma, which would be included within the grade 3 category. It is important to note that not all grade 3 lesions are a large B-cell lymphoma.
  • Current evidence shows that LYG is a disease of B cells.

OBJECTIVES:

  • To determine the response and long-term efficacy of alpha-Interferon in patients with lymphomatoid granulomatosis (LYG).
  • To determine the response and long-term efficacy of dose-adjusted (DA)-EPOCH-R chemotherapy in patients with grade 3 LYG or in patients who have failed interferon.

ELIGIBILITY:

  • Patients must have a tissue diagnosis of grade 1, 2 and/or 3 A/B LYG (or a diagnosis consistent with LYG) confirmed by the Laboratory of Pathology, NCI.
  • Patients with any stage of disease will be eligible.
  • Previously untreated and treated patients are eligible.
  • Patients age 12 or older will be eligible.
  • Female patients who are pregnant or lactating are not eligible
    Sponsor: National Cancer Institute (NCI)

    Current Primary Outcome: Overall response and long-term efficacy [ Time Frame: After completion of treatement ]

    Original Primary Outcome:

    Current Secondary Outcome:

    Original Secondary Outcome:

    Information By: National Institutes of Health Clinical Center (CC)

    Dates:
    Date Received: November 3, 1999
    Date Started: February 9, 1994
    Date Completion: February 1, 2022
    Last Updated: May 12, 2017
    Last Verified: March 27, 2017