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Primary CNS Lymphoma (Pcnsl)


Definition

  • Malignant lymphomas arising independently within the CNS without evidence of systemic lymphoma; most PCNSLs are aggressive B-cell lymphomas

Clinical Features

Epidemiology

  • Comprise between 1% to 5% of primary brain tumors

  • Peak incidence in immunocompetent individuals between 55 to 75 years of age

  • Slight male predilection: 3 : 2

  • Individuals with inherited or acquired immunodeficiency are predisposed to PCNSL

    • AIDS

    • Immunosuppression due to organ transplantation

    • Wiskott-Aldrich syndrome

  • Strong link to Epstein-Barr virus in immunocompromised patients with PCNSL

  • HAART therapy has decreased incidence of all non-Hodgkin lymphomas in AIDS patients

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