Báo cáo một trường hợp u lympho tế bào T/NK tại hạch với EBV dương tính=EBV-positive nodal T- and NK-cell lymphoma: a case report

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Tác giả: Hoàng Thiên Đặng, Đắc Quỳnh Anh Nguyễn, Oanh Thùy Linh Nguyễn, Ngọc Kim Ngân Tiêu

Ngôn ngữ: vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y học Việt Nam (Tổng hội Y học Việt Nam), 2024

Mô tả vật lý: tr.610-617

Bộ sưu tập: Metadata

ID: 478332

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In the 2022 International Consensus Classification of Mature Lymphoid Neoplasms and the 5th edition of the World Health Organization’s classification of hematolymphoid tumors, EBV-positive nodal T-/NK-cell lymphoma (EBV+ nPTCL) has been identified as a new provisional entity. This is a change from its previous classification as a variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS). EBV+ nPTCL is known for its aggressive clinical course, with a short median overall survival and no standard treatment. Therefore, it's important to distinguish EBV+ nPTCL from PTCL, NOS for appropriate treatment and prognosis. We present a case of EBV+ nPTCL in a 50-year-old female. Initially, the histological morphology and immunophenotype did not differ from other PTCL, NOS. However, the tumor cells tested positive for EBER and expressed cytotoxic markers, leading to the diagnosis of EBV+ nPTCL. This case highlights the significance of screening for EBER in PTCL.U lympho tế bào T/NK tại hạch với EBV dương tính (EBV+ nPTCL) là một phân nhóm mới trong phân loại đồng thuận quốc tế về u lympho năm 2022 (ICC 2022) và phiên bản thứ 5 của phân loại bệnh lý hệ tạo máu của Tổ chức Y tế Thế giới (WHO-HAEMS5). Trước đây, những trường hợp này được coi là biến thể của u lympho tế bào T ngoại biên, không đặc hiệu (PTCL, NOS). Tuy nhiên các nghiên cứu đã cho thấy EBV+ nPTCL là một thực thể riêng biệt, biểu hiện phân tử gây độc tế bào, tế bào u không điển hình về mặt hình thái, diễn tiến rất nhanh và không đáp ứng với hóa trị liệu thông thường. Vì vậy, EBV+ nPTCL cần được tách biệt với các PTCL, NOS khác để có chiến lược điều trị và tiên lượng bệnh phù hợp. Chúng tôi báo cáo một trường hợp EBV+ nPTCL ở một phụ nữ 50 tuổi. Hình thái mô học và kiểu hình miễn dịch ban đầu không khác biệt so với các PTCL, NOS khác. Tuy nhiên tế bào u dương tính với EBER và biểu hiện marker T gây độc hướng đến chẩn đoán EBV+ nPTCL. Trường hợp này nhấn mạnh tầm quan trọng của việc khảo sát EBER thường quy trên các trường hợp PTCL để tránh phân loại nhầm sang PTCL, NOS.

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