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International Journal of Hematology

, Volume 82, Issue 1, pp 63–65 | Cite as

Meningeal Lymphomatosis as the First Manifestation of Splenic Marginal Zone Lymphoma

  • Jordi Bruna
  • Sergio Martínez-Yelamos
  • Esther Alonso
  • Vicente Romagosa
  • Jordi Arruga
  • Susana Fernández
  • Alicia Domingo
  • Iñigo Rojas-Marcos
  • Josep Petit
  • Francisco Rubio
Case report

Abstract

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.

Key words

Meningeal lymphomatosis Splenic marginal zone lymphoma 

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Copyright information

© The Japanese Society of Hematology 2005

Authors and Affiliations

  • Jordi Bruna
    • 1
    • 5
  • Sergio Martínez-Yelamos
    • 1
  • Esther Alonso
    • 2
  • Vicente Romagosa
    • 4
  • Jordi Arruga
    • 3
  • Susana Fernández
    • 1
  • Alicia Domingo
    • 2
  • Iñigo Rojas-Marcos
    • 1
  • Josep Petit
    • 2
  • Francisco Rubio
    • 1
  1. 1.Departments of NeurologySpain
  2. 2.Departments of HaematologySpain
  3. 3.Departments of OphthalmologySpain
  4. 4.Departments of PathologySpain
  5. 5.Institut de Neuropatologia, Hospital Universitari de Bellvitge, Institut d’Investigació Biomèdica de BellvitgeUniversitat de BarcelonaBarcelonaSpain

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