Trial Information

Treatment and Natural History Study of Lymphomatoid Granulomatosis

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 (DA)-EPOCH-R chemotherapy in patients
with grade 3 LYG or in patients who have failed interferon.

To obtain tissue and blood to examine the immunologic phenotype, EBV viral loads and
molecular markers of clonality in patients with LYG.

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 to receive treatment but can
be followed for natural history.

DESIGN:

Interferon is used as initial treatment in patients with grades 1 and 2 LYG . Patients will
receive interferon for one year past CR.

Patients who progress after or during interferon, and patients with grade 3 LYG will receive
aggressive combination chemotherapy with DA-EPOCH-R (rituximab, etoposide, doxorubicin,
vincristine, cyclophosphamide and prednisone).

Patients who fail one treatment approach may be crossed over to the other.

A total of 85 patients will be enrolled at this single institution.