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History History
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Epidemiology Epidemiology
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Pathology Pathology
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Pathogenesis Pathogenesis
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Clinical Presentation Clinical Presentation
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Diagnostic Criteria Diagnostic Criteria
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Laboratory Testing Laboratory Testing
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Neuroimaging Neuroimaging
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Staging Staging
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Differential Diagnosis Differential Diagnosis
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Management Management
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Treatment Treatment
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Prognostic Factors Prognostic Factors
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Radiation Therapy Radiation Therapy
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Chemotherapy Chemotherapy
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Immunotherapy Immunotherapy
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High Dose Chemotherapy with Autologous Stem Cell Transplantation High Dose Chemotherapy with Autologous Stem Cell Transplantation
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Salvage Therapy Salvage Therapy
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Course and Prognosis Course and Prognosis
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Current Approach to Management Current Approach to Management
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References References
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133 Primary Central Nervous System Lymphoma
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Published:August 2016
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Abstract
The lymphoid nature of PCNSL was established unequivocally by modern immunohistochemical techniques. PCNSL has been associated with a variety of congenital (Wiskott-Aldrich syndrome, ataxia-telangiectasia) and acquired (human immunodeficiency virus [HIV], renal transplant recipients) immunodeficiency states. PCNSL tends to be supratentorial, periventricular, and involve the deep structures such as the basal ganglia. The Epstein-Barr virus (EBV) plays an important role in initiating the development of PCNSL in immunocompromised patients includinig those with HIV infection. Leukoencephalopathy is a serious complication of effective PCNSL treatment, but apparent only when the patient is in a durable remission. Treatment utilizes chemotherapy with or without radiation, which can cause more cognitive disability than chemotherapy, and autologous stem cell therapy is under investigation in selected patients.
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