Báo cáo ca lâm sàng thiếu máu tan máu tự miễn che lấp đa hồng cầu nguyên phát=Case report: polycythemia vera was covered by autoimmune hemolytic anemia

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Tác giả: Thị Thu Huyền Lê, Thanh Bình Nguyễn, Thị Thùy Trang Nguyễn, Thị Tuyết Nhung Phạm, Văn Hiệu Phạm, Thị Thanh Long 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.576-580

Bộ sưu tập: Metadata

ID: 478326

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Autoimmune hemolytic anemia is an anemia caused by shortened red blood cell life span due to the presence of autoantibodies against red blood cells. Polycythemia vera is a disease of excessive proliferation of red blood cells, belonging to the group of chronic malignant myeloproliferative diseases. Both diseases are diagnosed based on hemoglobin count, but they are opposites, autoimmune hemolytic anemia is characterized by hemoglobin <
120 g/L and immune hemolysis, while polycythemia vera is diagnosed when hemoglobin >
165 g/L in men and >
160 g/L in women accompanied by uncontrolled erythropoiesis due to mutations in the gene of the tyrosine kinase protein of the Janus kinase family (JAK) causing a decrease in the function of autoinhibition of cell reproduction. Doctors rarely have to distinguish between these two diseases and the probability of a patient being diagnosed with these two combined diseases is rare. We describe a clinical case of autoimmune hemolytic anemia, after control of autoimmune hemolytic anemia, polycythemia vera was revealed.Thiếu máu tan máu tự miễn là bệnh thiếu máu do đời sống của hồng cầu bị rút ngắn bởi sự xuất hiện tự kháng thể chống hồng cầu. Đa hồng cầu nguyên phát là bệnh tăng sinh quá mức dòng hồng cầu, thuộc nhóm các bệnh tăng sinh tủy mạn ác tính. Hai căn bệnh này đều được chẩn đoán dựa trên số lượng huyết sắc tố, nhưng chúng đối ngược nhau, thiếu máu tan máu tự miễn đặc trưng bởi lượng huyết sắc tố <
120 g/L và tình trạng tan máu miễn dịch, trong khi đa hồng cầu nguyên phát chẩn đoán khi lượng huyết sắc tố >
165 g/L với nam và >
160 g/L với nữ kèm theo tình trạng tăng sinh hồng cầu không kiểm soát do đột biến gen của protein tyrosine kinase thuộc Janus kinase family (JAK) gây giảm chức năng tự ức chế sinh sản tế bào. Ít khi các bác sĩ phải phân biệt hai bệnh lý này và xác suất một người bệnh được chẩn đoán hai bệnh lý kết hợp với nhau này là hiếm. Chúng tôi mô tả một trường hợp lâm sàng có thiếu máu tan máu tự miễn, sau khi kiểm soát được bệnh thiếu máu tan máu tự miễn đã bộc lộ bệnh đa hồng cầu nguyên phát.

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