Lymphomatoid granulomatosis. A case for aggressive therapy

Lymphomatoid granulomatosis is an uncommon disorder with features of vasculitis and lymphoma. Treatment has usually consisted of steroids and, occasionally, single cytotoxic drugs, but prognosis remains poor. The authors report a patient with life-threatening disease treated successfully with combination chemotherapy consisting of methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B) who achieved complete remission and remains disease-free over 3 years from diagnosis. Justification for an aggressive therapeutic approach to this disease is presented.