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阵发性睡眠性血红蛋白尿症(PNH)是一种获得性造血干细胞克隆性疾病,以发作性血管内溶血、静脉血栓形成、骨髓造血功能衰竭为主要表现。PNH干细胞存在克隆优势的机制目前仍不清楚。
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell clonal disease with episodic intravascular hemolysis, venous thrombosis, and bone marrow hematopoietic failure as the primary manifestation. The mechanism of the cloning advantage of PNH stem cells is still unclear.