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CASE REPORT
Fatal lymphomatoid granulomatosis with primary CNS-involvement in an immunocompetent 80-year-old woman
  1. David G Olmes1,
  2. Abbas Agaimy2,
  3. Stephan Kloska3,
  4. Ralf A Linker1
  1. 1Department of Neurology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany
  2. 2Institute of Pathology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany
  3. 3Department of Neuroradiology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany
  1. Correspondence to David Olmes, david.olmes{at}uk-erlangen.de

Summary

An 80-year-old woman presented with weight loss, fatigue, dizziness and a brain stem lesion. Extensive work-up revealed lymphomatoid granulomatosis (LYG) with primary clinical manifestation in the central nervous system (CNS), a rare Epstein-Barr virus-driven multisystem lymphoproliferative disorder, to be causative for the symptoms. Immunochemotherapy consisting of rituximab and temozolomide was started, but the disease progressed and the patient subsequently died. Histology, diagnostic criteria, differential diagnosis and treatment options for LYG with CNS involvement are discussed. This case demonstrates that LYG with CNS involvement may necessitate more aggressive treatment approaches than combination therapy with rituximab and temozolomide.

https://doi.org/10.1136/bcr-2014-206825

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