Аңдатпа
Relevance: Primary central nervous system (PCCNS) lymphomas are a rare type of lymphoma, accounting for 2% of all CNS lymphomas, and are associated with a poor prognosis. According to data from the Republic of Kazakhstan EROB Information System, in 2023-2024, the «CNS lymphoma» diagnosis was morphologically confirmed in 13 individuals. Among primary CNS lymphomas, the ALK-negative subtype of anaplastic large-cell T-cell lymphoma is a highly malignant tumor with an aggressive clinical course. Treatment of such patients remains challenging, requiring an expanded evidence base and more clinical case reports.The study aimed to demonstrate the effectiveness of combined chemo-targeted therapy with autologous bone marrow transplantation in a patient with ALK-negative anaplastic large cell CNS lymphoma (a T-cell lymphoma subtype) through a clinical case and literature review.Methods: This article presents a literature review and a clinical case of a patient with T-cell lymphoma of the central nervous system. Diagnostic assessments included computed tomography (CT), positron emission tomography (PET), magnetic resonance imaging (MRI) of the brain, as well as histopathological and immunohistochemical examination of postoperative tissue samples. The disease course and response to treatment are described.Results: A patient with a provisional clinical diagnosis of «Primary anaplastic CNS lymphoma, ALK-negative subtype» underwent microsurgical tumor resection. Given the rare nature and localization of the tumor, a histopathological re-evaluation with immunohistochemical analysis of the postoperative specimen was performed. A therapeutic strategy was selected, including using the targeted agent brentuximab vedotin. This case illustrates the potential of combined chemo-targeted therapy in treating ALK-negative anaplastic CNS lymphoma, taking into account the tumor’s biological characteristics and the patient’s individual features.Conclusion: The correct choice of treatment strategy depends on timely and accurate diagnosis, making diagnostic workup - including morphological and immunohistochemical evaluation - a key step in patient management. In recent years, the strategy of choice for improving prognosis and survival in such patients has been developing and implementing combined therapeutic approaches, incorporating both intensive chemotherapy regimens and modern targeted therapies.