Doukas M A, Fleming D, Jennings D
Veterans Affairs Medical Center, Hematology/Oncology Section, Lexington, KY 40511-1093, USA.
Bone Marrow Transplant. 1998 Oct;22(7):717-21. doi: 10.1038/sj.bmt.1701345.
Paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal hematopoietic disorder characterized by protean clinical manifestations, is associated with significant morbidity and mortality. We report a 24-year-old patient with PNH complicated by deep vein thrombosis who underwent syngeneic bone marrow transplantation. No clinical symptomatology or stigmata of disease have recurred. Immunophenotyping of this patient over 12 years after her procedure revealed all peripheral circulating cells to express normal levels of CD59. Histocompatible marrow transplantation remains the definitive method of treatment for PNH with modern immunophenotyping capable of sensitive follow-up post-transplant.
阵发性睡眠性血红蛋白尿（PNH）是一种获得性克隆性造血疾病，临床表现多样，与显著的发病率和死亡率相关。我们报告一名24岁患有PNH并伴有深静脉血栓形成的患者，该患者接受了同基因骨髓移植。目前尚无疾病的临床症状或体征复发。对该患者术后12年的免疫表型分析显示，所有外周循环细胞均表达正常水平的CD59。组织相容性骨髓移植仍然是治疗PNH的决定性方法，现代免疫表型分析能够在移植后进行敏感的随访。
