Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of extranodal non-Hodgkin's lymphoma (NHL) involving the brain, leptomeninges, or eyes. PCNSL typically remains confined to the nervous system. The disease is commonly diagnosed by stereotactic brain biopsy and the diagnostic process typically consumes the tissue. An increased risk of lymphoproliferative diseases such as PCNSL is seen in organ transplant recipients who receive immunosuppressive drugs. However, the increased risk of PCNSL in allograft recipients is observed mainly in patients who received older, azathioprine-based immunosuppressive regimens that are not commonly used today and is less common in patients treated with cyclosporine-based therapy. A better biological understanding of PCNSL enables the development of customized treatment approaches for this patient population. However, multicenter collaboration has resulted in the first gene expression profile study, and other study of biomarkers of prognosis, which have been published. Although there is a large gap between the understanding of other forms of extranodal B-cell lymphomas and PCNSL, it is anticipated that this gap will close in the coming years as multicenter collaboration, tissue preservation methods, and molecular techniques are improved and refined.
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