Meningeal Lymphomatosis as the First Manifestation of Splenic Marginal Zone Lymphoma

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.

This is a preview of subscription content, log in to check access.

References

  1. 1.

    Franco V, Florena AM, Iannitto E. Splenic marginal zone lym-phoma.Blood. 2003;101:2464–2472.

    Article  PubMed  CAS  Google Scholar 

  2. 2.

    Troussard X, Valensi F, Duchayne E, et al. Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients.Br J Haematol. 1996;93:731–736.

    Article  PubMed  CAS  Google Scholar 

  3. 3.

    Chacòn JI, Mollejo M, Muñoz E, et al. Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients.Blood. 2002;100:1648–1654.

    PubMed  Google Scholar 

  4. 4.

    Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes/splenic marginal-zone lymphoma.Semin Hematol. 1999;36:148–154.

    PubMed  CAS  Google Scholar 

  5. 5.

    Demopoulos A, DeAngelis L. Neurologic complications of leukemia.Curr Opin Neurol. 2002;15:691–699.

    Article  PubMed  Google Scholar 

  6. 6.

    Morrison C, Shah S, Flinn IW. Leptomeningeal involvement in chronic lymphocytic leukemia.Cancer Pract. 1998;6:223–228.

    Article  PubMed  CAS  Google Scholar 

  7. 7.

    Voght-Schaden M, Wildemann B, Stellejer M, Hans J, Storch-Hagenlocher B, Ginshach C. Leptomeningeal dissemination of chronic lymphatic leukemia: molecular genetic detection in cerebrospinal fluid [in German].Nervenarzt. 1999;70:363–367.

    Article  Google Scholar 

  8. 8.

    Wang ML, Shih LY, Dunn P, Kuo MC. Meningeal involvement in B-cell chronic lymphocytic leukemia: report of two cases.J Formos Med Assoc. 2000;99:775–778.

    PubMed  CAS  Google Scholar 

  9. 9.

    Poltorak M, Czlonkowska A, Nowicka K. Chronic lymphocytic leukemia: study of cell subsets in cerebrospinal fluid and peripheral blood.Eur Neurol. 1983;22:289–292.

    Article  PubMed  CAS  Google Scholar 

  10. 10.

    Miller K, Budke H, Orazi A. Leukemic meningitis complicating early stage chronic lymphocytic leukemia.Arch Pathol Lab Med. 1997;121:524–527.

    PubMed  CAS  Google Scholar 

  11. 11.

    Brick WG, Majmundar M, Hendricks K, Kallab AM, Burguess RE, Jilella AP. Leukemic leptomeningeal involvement in stage 0 and stage 1 chronic lymphocytic leukemia.Leuk Lymphoma. 2002;43: 199–120.

    Article  PubMed  CAS  Google Scholar 

  12. 12.

    Weizsaeker M, Koelmel HW. Meningeal involvement in leukemias and malignant lymphomas of adults: incidence, course of disease, and treatment for prevention.Acta Neurol Scand. 1979;60:363–370.

    Article  Google Scholar 

  13. 13.

    Navarrete D, Bodega E. Leukemic meningitis in a patient with hairy cell leukemia: a case report.Nouv Rev Fr Hematol. 1987;29: 247–249.

    PubMed  CAS  Google Scholar 

  14. 14.

    Knecht H, Budmiger H, Groscurth P, Streuli RA. Central nervous system involvement in hairy cell leukemia.Klin Wochenschr. 1985; 63:423–427.

    Article  PubMed  CAS  Google Scholar 

  15. 15.

    Wolfe DW, Scopelliti JA, Boselli BD. Leukemic meningitis in a patient with hairy cell leukemia: a case report.Cancer. 1984;54: 1085–1087.

    Article  PubMed  CAS  Google Scholar 

  16. 16.

    Bilgrami S, Shafi N, Pesanti EL, et al. Mantle-cell lymphoma in a patient with human immunodeficiency viral infection.Acta Haematol. 1995;93:101–104.

    Article  PubMed  CAS  Google Scholar 

  17. 17.

    Lunning MA, Stetler-Stevenson M, Silverstein PT, Zenger V, Marti GE. Spontaneous (pathological) splenic rupture in a blastic variant of mantle cell lymphoma: a case report and literature review.Clin Lymphoma. 2002;3:117–120.

    Article  PubMed  Google Scholar 

  18. 18.

    Gotlib V, Singareddy S, Gergis U, et al. Leptomeningeal involvement in a patient with splenic lymphoma with villous lymphocytes.Leuk Lymphoma. 2002;43:1337–1340.

    Article  Google Scholar 

  19. 19.

    Yamazaki K, Shimizu S, Negami T, et al. Leukemic meningitis in a patient with splenic lymphoma with villous lymphocytes (SLVL).Cancer. 1994;73:61–65.

    Article  Google Scholar 

  20. 20.

    Marmont AM. Leukemic meningitis in B-cell chronic lymphocytic leukemia: resolution following intrathecal methotrexate.Blood. 2000;9:776–777.

    Google Scholar 

  21. 21.

    De Rossi G, Zarcone D, Muro F, et al. Adhesion molecule expression on B-cell chronic lymphocytic leukemia cells: malignant cell phenotypes define distinct disease subsets.Blood. 1993;81: 2679–2687.

    Google Scholar 

  22. 22.

    Csanaky G, Matutes E, Vass JA, Monilla R, Catovsky D. Adhesion receptors on peripheral blood leukemic B cells: a comparative study on B cell chronic lymphocytic leukemia and related lym-phoma/leukemias.Leukemia. 1997;11:408–415.

    Article  CAS  Google Scholar 

  23. 23.

    Strazielle N, Ghersi-Egea JF. Choroid plexus in the central nervous system: biology and physiopathology.J Neuropathol Exp Neurol. 2000;59:561–574.

    Article  CAS  Google Scholar 

  24. 24.

    Steffen B J, Breier G, Butcher EC, Schulz M, Engelhardt B. ICAM-1, VCAM-1 and MAdCAM-1 are expressed on choroid plexus epithelium but not endothelium and mediate binding of lymphocytes in vitro.Am J Pathol. 1996;148:1819–1838.

    PubMed Central  PubMed  CAS  Google Scholar 

  25. 25.

    Baehring JM, Damek D, Martin EC, Batensky RB, Hochberg FH. Neurolymphomatosis.Neuro-Oncol. 2003;5:104–115.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Affiliations

Authors

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.

About this article

Cite this article

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). https://doi.org/10.1532/IJH97.E0501

Download citation

Key words

  • Meningeal lymphomatosis
  • Splenic marginal zone lymphoma