阵发性夜间血红蛋白尿(PNH)是一种复杂的干细胞疾病,表现为:溶血性贫血,伴有血红蛋白血症和血红蛋白尿;少见的静脉血栓形成以及上腹和背部严重的发作性疼痛。某些病例骨髓显示相对或绝对的再生不良,导致网织红细胞、白细胞和血小板减少 .在罕见病例中,这种综合征最后可成为急性粒细胞白血病。 Paroxysmal nocturnal hemoglobinuria (PNH) is a complex stem cell disorder characterized by hemolytic anemia associated with hemoglobinemia and hemoglobinuria; rare venous thrombosis and severe episodes of pain in the upper abdomen and back. In some cases, bone marrow shows a relative or absolute poor regeneration, resulting in a decrease in reticulocytes, leukocytes, and thrombocytopenia, and in rare cases this syndrome can eventually become acute myeloid leukemia.