The 26 patients ranged in age from 6 to 68 years (median 42 years). The
lymphoproliferative disorder was diagnosed from 1 to 211 months (median 80 months) after
transplantation. The type of transplant was kidney (n = 21), heart or heart-lung (n = 4), or liver (n = 1). Most patients received
azathioprine and
prednisone, in addition to
antilymphocyte globulin or
cyclosporine, for post-transplant immunosuppression. Eight patients had
lymphoma that could be classified according to the International Working Formulation (IWF-F, IWF-G, IWF-H). Sixteen patients had polymorphic
lymphoma, and 2 patients were classified as having polymorphic lymphoid
hyperplasia. Patients were staged by the Ann Arbor staging system. Nine patients had stage I disease, 4 stage II, 6 stage III, and 7 stage IV. Central nervous system, lung, or marrow involvement was present in 27%, 23%, and 14% of patients, respectively. In the 17 patients studied, immunophenotype was monoclonal B-cell (n = 12), malignant T-cell (n = 2), or polyclonal B-cell (n = 3). The initial therapeutic approach was generally a reduction in immunosuppression, but, thereafter, the approach to
therapy varied. In patients with localized disease, surgical excision and/or involved field
radiotherapy were utilized as applicable. For patients with more extensive disease, other approaches such as high-dose
acyclovir,
combination chemotherapy, or
alpha interferon were utilized. Overall, 15 of 26 patients (58%) responded to systemic
therapy or were rendered disease-free either by surgery or radiation, including 8 (31%) with a complete remission (CR). Only 3 of 9 patients responded to
chemotherapy, whereas 4 of 13 patients responded to
acyclovir (including 3 patients who experienced CR). Remission duration ranged from 8 to 122 months (median 32+ months). Twenty-one of 26 patients (81%) have died. Survival ranged from less than 1 to 122 months (median 14 months).
CONCLUSION: