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阵发性睡眠性血红蛋白尿(paroxysmalnocturnal hemoglobinuria,PNH)系一复杂的干细胞疾病,临床上表现为溶血性贫血伴有血红蛋白血症和血红蛋白尿。静脉血栓形成和严重的腹、背部疼痛则较少见。一些患者有相对或绝对的骨髓增生低下,故网织红细胞、白细胞及血小板均可减少。罕见有最终演变为急性粒细胞白血病者。PNH 的根本原因是骨髓增生异常,形成一个累及外周血3系细胞的异常细胞群体。该群体细胞能特异地固定补体,其细胞膜对补体又异常敏感。补体激活在红细胞引起溶血,在
Paroxysmal nocturnal hemoglobinuria (PNH) is a complex stem cell disease that is clinically manifest as hemolytic anemia associated with hemoglobinemia and hemoglobinuria. Venous thrombosis and severe abdominal and back pain are less common. Some patients have relative or absolute low myeloproliferation, so reticulocytes, white blood cells and platelets can be reduced. Rare have eventually evolved into acute myeloid leukemia. The underlying cause of PNH is myelodysplasia, which forms an abnormal cell population that affects peripheral blood 3 lineage cells. The population of cells can specifically fix complement, the cell membrane is extremely sensitive to complement. Complement activation causes hemolysis in erythrocytes, at