Primary Central Nervous System Posttransplant Lymphoproliferative Disorders

Abstract

Posttransplant lymphoproliferative disorders (PTLDs) represent a spectrum ranging from Epstein-Barr virus (EBV)-driven polyclonal lymphoid proliferations to EBV+ or EBV- malignant lymphomas. Central nervous system (CNS) PTLDs have not been characterized fully. We reviewed the clinical, radiologic, and pathologic features of 12 primary CNS PTLDs to define them more precisely. Patients included 10 males and 2 females (median age, 43.4 years) who were recipients of kidney (n = 5), liver (n = 2), heart (n = 2), peripheral blood stem cells (n = 2), or bone marrow (n = 1). All received immunosuppressive therapy. CNS symptoms developed 3 to 131 months (mean, 31 months) after transplantation. By neuroimaging, most showed multiple (3 to 9) intra-axial, contrast-enhancing lesions. Histologic sections showed marked expansion of perivascular spaces by large, cytologically malignant lymphoid cells that were CD45+, CD20+, EBV+ and showed light chain restriction or immunoglobulin gene rearrangement. In distinction to PTLDs in other organ systems, CNS PTLDs were uniformly high-grade lymphomas that fulfilled the World Health Organization criteria for monomorphic PTLDs. Extremely short survival periods were noted for each CNS PTLD that followed peripheral blood stem cell transplantation. Survival of others with CNS PTLD varied; some lived more than 2 years.

Primary central nervous system post-transplant lymphoproliferative disorders following allogeneic hematopoietic stem cell transplantation

“…Primary CNS-PTLD following allo-HSCT presents a particular challenge as there is limited clinical experience, few reported cases and lack of prospective studies. Only a small number of cases isolated CNS-PTLD following allo-HSCT have been reported [10][11][12][13][14][15][16][17][18] (Table 1). The latency between transplantation and development of CNS-PTLD ranged from 2 to 23 months.…”

Post-transplant lymphoproliferative disorder localized in the colon after liver transplantation: Report of a case

“…Extranodal disease and visceral nodal involvement are characteristic. 1,14 Patients who have malignant PTLD often also have fever, weight loss, fatigue, and lymphadenopathy, and mass effects from the tumor itself, often in the central nervous system, 15 gastrointestinal tract, and head and neck. Post-transplant lymphoproliferative disorder frequently involves the transplanted organ itself, 9,16 and allograft dysfunction is a common presentation.…”

Extradural Thoracic Spinal Cord Compression: Unusual Initial Presentation of Post-transplant Lymphoproliferative Disorder

“…2 The risk of PTLD development is significantly higher in the early posttransplant period (Ͻ1 year after transplantation), especially in heart-lung and lung transplant recipients. Primary central nervous system (CNS) localization is observed in about 5% to 15% of all PTLD cases, 7 and almost invariably affects the brain. This is in contrast with patients with "conventional" non Hodgkin's lymphoma, in which the CNS is less frequently affected.…”

“…7 PTLD treatment always consists of reduced immunosuppression. Early lesions, including plasma cell hyperplasia and lesions resembling infectious mononucleosis, are usually polyclonal and often regress only after reduction of immunosuppression.…”