Xơ tủy nguyên phát: cập nhật về chẩn đoán và phân nhóm nguy cơ – năm 2024=Primary myelofibrosis: update 2024 on diagnosis and risk classification

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Tác giả: Thúy Hà Châu, Thị Xinh Phan

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.395-404

Bộ sưu tập: Metadata

ID: 478291

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Primary myelofibrosis (PMF) is a disorder of myeloproliferative neoplasm. The disease is characterized by anemia, splenomegaly, hepatomegaly, and bone marrow fibrosis. Gene mutations including JAK2, CALR, MPL, ASXL1, EZH2, TET2,... play an important role in the molecular pathogenesis of the PMF. The PMF is divided into 2 stages: pre-PMF and overt PMF, diagnosed according to WHO 2016 and updated according to WHO 2022 criterias. The pre-PMF stage easily overlaps with essential thrombocythemia. The diagnostic criteria of WHO 2022 are no different from WHO 2016. The marrow biopsy technique is of primary value in the differential diagnosis of pre-PMF and essential thrombocytosis based on the morphology of platelets in the bone marrow. In addition, this is a heterogeneous group of diseases, which are prognosticated based on multiple prognostic classification systems including IPSS, DIPSS, DIPSS plus, MIPSS70, MIPSSv2 and GIPSS systems. Based on the prognostic classification, patients with the high risk group have a chance to receive treatment and early stem cell transplantation which contribute to improving the prognosis.Xơ tủy nguyên phát là một thể bệnh thuộc nhóm tân sinh tăng sinh tủy. Bệnh đặc trưng bởi thiếu máu, lách to, gan to và xơ hóa tủy xương. Các đột biến gen gồm JAK2, CALR, MPL, ASXL1, EZH2, TET2,... có vai trò quan trọng trong sinh bệnh học phân tử của bệnh. Bệnh được chia thành 2 giai đoạn là tiền xơ tủy và xơ tủy toàn phát, chẩn đoán dựa theo tiêu chuẩn của WHO năm 2016 và được cập nhật theo WHO 2022. Điểm khác biệt trong tiêu chuẩn chẩn đoán của WHO 2016 so với trước đây là có tiêu chuẩn chẩn đoán giai đoạn tiền xơ tủy, mà giai đoạn này rất dễ trùng lắp với bệnh cảnh tăng tiểu cầu nguyên phát. Tiêu chuẩn chẩn đoán của WHO 2022 không khác biệt so với WHO 2016. Kỹ thuật sinh thiết tủy có giá trị tiên quyết trong chẩn đoán phân biệt tiền xơ tủy và tăng tiểu cầu nguyên phát dựa vào hình thái mẫu tiểu cầu trong tủy xương. Ngoài ra, đây là một nhóm bệnh không đồng nhất, được phân nhóm tiên lượng dựa vào nhiều hệ thống phân nhóm tiên lượng gồm hệ thống IPSS, DIPSS, DIPSS plus, MIPSS70, MIPSSv2 và GIPSS. Dựa vào việc phân nhóm tiên lượng, các bệnh nhân thuộc nhóm nguy cơ cao được điều trị tích cực và tiến hành ghép tế bào gốc sớm góp phần cải thiện tiên lượng.

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