The diagnosis of lymphoma of the CNS rests on careful attention to clinical signs and symptoms but has certainly been facilitated by advances in neuroimaging and CSF laboratory analysis. The most common form of CNS involvement in NHL is leptomeningeal disease. Spinal epidural disease may present as an emergency with spinal cord compression, and parenchymal brain metastases may cause seizures, headache, or focal signs and symptoms. After a diagnosis is established, the use of intrathecal or systemic chemotherapy and radiotherapy can improve survival and palliate symptoms. High-dose systemic chemotherapy with hematopoietic stem cell transplantation is an important treatment option at CNS relapse of NHL and for primary CNS lymphoma. An important consideration in this setting is the treatment of CNS disease before transplantation. The prognosis for disease-free survival and cure is much better for patients who have treatment and clearing of the CNS before transplantation than for patients who have active CNS disease at the time of transplant.
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