Journal of Neuroimmunology
Volume 218, Issue 1 , Pages 125-128, 25 January 2010

An atypical case of X-linked lymphoproliferative disease revealed as a late cerebral lymphoma

  • B. Hervier

      Affiliations

    • Internal Medicine Department, CHU NANTES, Place Alexis Ricordeau, 44093 Nantes, France
    • Corresponding Author InformationCorresponding author. Tel.: +33 2 40 08 33 55; fax: +33 2 40 8 33 79.
    • ,
  • S. Latour

      Affiliations

    • Unité Inserm U768, Hopital Necker-Enfants-Malades, 75015 Paris, France
    • ,
  • D. Loussouarn

      Affiliations

    • Pathology Department, CHU NANTES, France
    • ,
  • M. Rimbert

      Affiliations

    • Laboratory of Immunology, CHU NANTES, France
    • ,
  • G De-Saint-Basile

      Affiliations

    • Unité Inserm U768, Hopital Necker-Enfants-Malades, 75015 Paris, France
    • ,
  • C. Picard

      Affiliations

    • Unité Inserm U768, Hopital Necker-Enfants-Malades, 75015 Paris, France
    • ,
  • M. Hamidou

      Affiliations

    • Internal Medicine Department, CHU NANTES, Place Alexis Ricordeau, 44093 Nantes, France

Received 3 August 2009; received in revised form 12 October 2009; accepted 19 October 2009. published online 11 November 2009.

Abstract 

X-linked lymphoproliferative disease (XLP) is an inherited immunodeficiency, partially characterized by a defect in cytotoxicity to Epstein-Barr virus. This viral infection is therefore often fatal in affected boys, whilst a variety of immune disorders or proliferative diseases may occur in surviving patients.

We report an atypical case of a 41year-old male who presented with a primitive B-cell cerebral lymphoma, revealing an XLP.

This presentation was unusual because of its late onset, the broad spectrum of the familial characteristics, its initial presentation as a cerebral lymphoma, and the occurrence of B-cell alymphocytosis associated with a-gamma-globulinemia.

Keywords: Cerebral lymphoma, Immune defect, X-linked lymphoproliferative disease

To access this article, please choose from the options below

Login to an existing account or Register a new account.

  • Purchase this article for 31.50 USD (You must login/register to purchase this article)

    Online access for 24 hours. The PDF version can be downloaded as your permanent record.

  • Subscribe to this title

    Get unlimited online access to this article and all other articles in this title 24/7 for one year.

  • Claim access now

    For current subscribers with Society Membership or Account Number.

  • Visit SciVerse ScienceDirect to see if you have access via your institution.
 

PII: S0165-5728(09)00404-4

doi:10.1016/j.jneuroim.2009.10.012

Journal of Neuroimmunology
Volume 218, Issue 1 , Pages 125-128, 25 January 2010

Article Tools

* Image Usage & Resolution