Meningeal Lymphomatosis as the First Manifestation of Splenic Marginal Zone Lymphoma


Meningeal lymphomatosis (ML) as the first manifestation of a splenic marginal zone lymphoma (SMZL) is rare. The descriptions of only 2 cases with this complication, one of which had ML as the first manifestation, have been published to date. We describe a 53-year-old man, an ex-smoker, who presented with transitory episodes of bilateral loss of visual acuity. On examination, only papilledema and splenomegalia were observed. The hemogram showed a predominance of lymphocytes with a villous morphology. Cytochemical staining and an immunophenotypic analysis revealed a positive reaction to tartrate-sensitive acid phosphatase and B-lineage markers (CD19+, CD20+, CD79b+, surface immunoglobulin κ expression, immunoglobulin D+, CD5-, CD23-, CD10-, CD25-, CD103-, and CD11c-). Magnetic resonance imaging of the brain showed tumoral infiltration in both optic nerves and in the cervicodorsal meninges. The cerebrospinal fluid examination revealed significant pleocytosis, and all lymphocytes had a phenotype identical to that of the peripheral blood, confirming the presence of ML. The bone marrow section also showed lymphocytes with an immunophenotype identical to that of the peripheral blood. A splenectomy confirmed the SMZL diagnosis. Treatment with corticosteroids and intrathecal chemotherapy was administrated; however, the response was not good, and the patient died. In this report, we discuss the other 2 cases and ML in B-cell chronic lym-phoproliferative disorders.

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Corresponding authors

Correspondence to Jordi Bruna or Sergio Martínez-Yelamos or Esther Alonso or Vicente Romagosa or Jordi Arruga or Susana Fernández or Alicia Domingo or Iñigo Rojas-Marcos or Josep Petit or Francisco Rubio.

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Bruna, J., Martínez-Yelamos, S., Alonso, E. et al. Meningeal Lymphomatosis as the First Manifestation of Splenic Marginal Zone Lymphoma. Int J Hematol 82, 63–65 (2005).

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Key words

  • Meningeal lymphomatosis
  • Splenic marginal zone lymphoma