Posttransplant Lymphoproliferative Disorder: Significance of Central Nervous System Involvement

• Published in: Transplantation proceedings Vol. 37; no. 2; pp. 954 - 955
• Main Authors: Buell, J.F., Gross, T.G., Hanaway, M.J., Trofe, J., Roy-Chaudhury, P., First, M.R., Woodle, E.S.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: New York, NY Elsevier Inc 01-03-2005; Elsevier Science
• Subjects: Adult; Biological and medical sciences; Central Nervous System - immunology; Child; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Hematologic and hematopoietic diseases; Humans; Kidney Transplantation - immunology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoproliferative Disorders - epidemiology; Medical sciences; Postoperative Complications - immunology; Registries; Retrospective Studies; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tissue, organ and graft immunology; United States; United States; Medicine; Treatment; Posttransplant lymphoproliferative disorder; Surgery; Lymphoproliferative syndrome; Central nervous system; Graft; Malignant hemopathy; Transplantation
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2004.12.130

Few data exist regarding central nervous system (CNS) involvement in patients with posttransplant lymphoproliferative disorder (PTLD). The purpose of this study was to review the Israel Penn International Transplant Tumor Registry (IPITTR) experience with CNS involvement by PTLD.
Nine hundred ten PTLD cases from the United States were reported to the IPITTR and reviewed for CNS involvement.
One hundred thirty-six transplant recipients with PTLD (15%) had CNS involvement. The highest incidence of CNS involvement occurred in pancreas (3 of 11; 27%) and kidney transplant recipients (76 of 429; 18%). Fifteen cases occurred in children and 121 cases in adults. For both children and adults, isolated CNS disease was associated with better survival when compared with multiple-site involvement (children: 29% vs 0%; adults: 12% vs 6%;
P < .05). Three-year survival in PTLD patients with CNS involvement was 9.4% and without CNS involvement was 49.4% (
P < .01). Radiation therapy alone appeared to provide the best survival rates (25%).
CNS involvement in transplant recipients with PTLD carries an ominous prognosis; however, isolated CNS involvement has a better prognosis than CNS plus extracranial involvement. Radiation therapy alone provides the best results, but this may be a reflection of isolated CNS disease.