HemaSphere (Aug 2023)

Treatment Strategies and Prognostic Factors in Secondary Central Nervous System Lymphoma: A Multicenter Study of 124 Patients

  • Hannes Treiber,
  • Verena Nilius-Eliliwi,
  • Nicole Seifert,
  • Deepak Vangala,
  • Meng Wang,
  • Sabine Seidel,
  • Thomas Mika,
  • Dominik Marschner,
  • Vanja Zeremski,
  • Rebecca Wurm-Kuczera,
  • Leandra Caillé,
  • Claudia I. Chapuy,
  • Lorenz Trümper,
  • Thomas Fischer,
  • Michael Altenbuchinger,
  • Gerald G. Wulf,
  • Gerald Illerhaus,
  • Sascha Dietrich,
  • Roland Schroers,
  • Björn Chapuy

DOI
https://doi.org/10.1097/HS9.0000000000000926
Journal volume & issue
Vol. 7, no. 8
p. e926

Abstract

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Secondary central nervous system lymphoma (SCNSL) is a rare and difficult to treat type of Non-Hodgkin lymphoma characterized by systemic and central nervous system (CNS) disease manifestations. In this study, 124 patients with SCNSL intensively treated and with clinical long-term follow-up were included. Initial histopathology, as divided in low-grade, other aggressive, and diffuse large B-cell lymphoma (DLBCL), was of prognostic significance. Overall response to induction treatment was a prognostic factor with early responding DLBCL-SCNSL in comparison to those non-responding experiencing a significantly better progression-free survival (PFS) and overall survival (OS). However, the type of induction regime was not prognostic for survival. Following consolidating high-dose chemotherapy and autologous stem cell transplantation (HDT-ASCT), DLBCL-SCNSL patients had better median PFS and OS. The important role of HDT-ASCT was further highlighted by favorable responses and survival of patients not responding to induction therapy and by excellent results in patients with de novo DLBCL-SCNSL (65% long-term survival). SCNSL identified as a progression of disease within 6 months of initial systemic lymphoma presentation represented a previously not appreciated subgroup with particularly dismal outcome. This temporal stratification model of SCNSL diagnosis revealed CNS progression of disease within 6 months as a promising candidate prognosticator for future studies.