Plasmacytic post-transplant lymphoproliferative disorder: a case series of nine patients

Abstract

SummaryPost-transplant lymphoproliferative disorder (PTLD) is a serious complication of organ transplantation. Although PTLD typically has a B-cell histology, an uncommon variant, plasmacytic PTLD can present as a monoclonal plasma cell proliferation similar to plasmacytomas seen in multiple myeloma. A retrospective analysis was performed on nine patients at our center with plasmacytic PTLD as characterized by plasmacytic histology with the presence of CD138 and lack of CD20. Of the 210 adult solid organ transplant PTLD patients diagnosed between 1988 and 2012, 9 (4%) had a histological appearance consistent with plasmacytic PTLD. The median time from transplant to diagnosis was 3.7 years (range 8 months-24 years). All patients presented with extranodal and often subcutaneous solid tumors. Laboratory features included elevated LDH and beta-2 microglobulin levels, monoclonal gammopathy, and EBV positivity of the tumor. Unlike conventional multiple myeloma, patients had normal calcium levels and only mild anemia. Six patients who have completed treatment achieved complete responses with radiation therapy and/or reduction in immunosuppression with two patients now greater than 5 years in continuous complete response. Plasmacytic PTLD, despite its plasmacytic histology, is responsive to conventional therapies used for B-cell PTLD including reduction in immunosuppression and radiation therapy.

Successful treatment with lenalidomide of secondary multiple myeloma with extramedullary liver plasmacytoma in a renal transplant recipient: A case report and review of the literature

“…In the present case report, the patient developed nasal plasmacytoma 7 years following renal transplantation, and experienced relapsed MM with extramedullary liver plasmacytoma 4 years subsequent to this. The patient did not exhibit a large number of abnormal plasma cells in the bone marrow cells, in agreement with previous studies (5,20,22,23). In this case, deceased donor and EBV infection are the 2 major association factors for the post-transplantation PCN observed in the patient.…”

“…Plasmacytoma is a tumor of terminally differentiated monoclonal plasma cells, and a frequent complication of MM, appearing at diagnosis or during disease progression (5,(20)(21)(22)(23). Post-transplant plasmacytomas have been described at several sites, including the allograft, skin, peritoneum, gastrointestinal tract and gingiva.…”

“…In contrast to classical PTLDs, there are limited studies on plasma cell malignancies that occur following solid organ transplantation. Multiple myeloma (MM) represents ≤4% of all PTLDs, and is associated with a poor response to discontinuation of immunosuppression and conventional therapy, and a short median survival rate (4,5). Generally, the presence of plasma cells in the liver is associated with aggressive forms of MM (6,7).…”

Enfermedad linfoproliferativa postrasplante de órgano sólido

“…Los hallazgos de dos series de casos de adultos receptores de trasplante de órgano sólido publicadas por Trappe y colaboradores (46) y Karuturi y colaboradores (47), que incluyeron 8 y 9 pacientes, respectivamente, mostraron características muy similares como la positividad para CD138, la negatividad para CD20, la restricción de cadenas livianas, la presencia de paraproteína sérica y la asociación con VEB en 10 de los 17 casos (58,8 %). Son raros el compromiso de la médula ósea y las lesiones líticas en hueso, mientras que los plasmacitomas extranodales, principalmente de localización subcutánea, estuvieron presentes en todos ellos.…”

Classification and Treatment of Posttransplant Lymphoproliferative Disorders

“…Plasmacytic PTLD can benefit from conventional radiotherapy or chemotherapy that is effective in lymphoma. 52 In a pediatric population, an early-onset, EBV-negative form of plasmacytic PTLD was shown to respond well to dexamethasone or thalidomide.…”