See ((CNS lymphoma))
Primary central nervous system lymphoma (PCNSL) is an invasive malignant lymphoma confined to the central nervous system.
Although patients undergoing first-line treatment can achieve complete response, most of them still relapse within two years.
Relapsed lymphoma is derived from occult lymphoma cells, and B cell receptor pathway activation and immune escape are the key mechanisms for the pathogenesis of PCNSL.
Most relapses are in the central nervous system, a small number of relapses are isolated systemic relapses, and clinical symptoms occur early and vary.
Current treatments for relapse include high-dose methotrexate rechallenge and other regimens of chemotherapy, whole-brain radiation therapy, hematopoietic stem-cell transplantation, targeted therapy and immunotherapy, which have become promising treatments.
The overall prognosis of relapsed PCNSL is very poor, although it is affected by many factors.
Primary central nervous system lymphoma (PCNSL) is a highly malignant non-Hodgkin’s lymphoma (NHL) that originates in the central nervous system (CNS) and eyes.
Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of PCNSL, followed by lymphoma of T cell or natural killer cell origin.
PCNSL accounts for 2-3% of NHL and 4% of CNS malignancies.
Despite significant improvements in the management of PCNSL, up to 60% of patients eventually relapse, and there is currently no accepted standard salvage regimen, additionally, the rate of remission is low, while prognosis after relapse remains poor.