Primary Central Nervous System Posttransplant Lymphoproliferative Disorders

Amilcar A. Castellano-Sanchez*, Shiyong Li, Jiang Qian, Anand Lagoo, Edward Weir, Daniel J. Brat

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

120 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)246-253
Number of pages8
JournalAmerican Journal of Clinical Pathology
Volume121
Issue number2
DOIs
StatePublished - Feb 2004

Keywords

  • Brain tumor
  • Epstein-Barr virus
  • Immunosuppression
  • Lymphoma
  • Posttransplant lymphoproliferative disorder
  • PTLD
  • Transplantation

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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