Serin Istemi, Bayir Aslıhan, Goze Hasan, Yokus Osman
Department of Hematology, University of Health Science, Istanbul Training & Research Hospital, Istanbul, Turkey.
Department of Internal Medicine, University of Health Science, Bagcilar Training & Research Hospital, Istanbul, Turkey.
Int J Hematol Oncol. 2020 Nov 13;10(1):IJH30. doi: 10.2217/ijh-2020-0013.
Paroxysmal nocturnal hemoglobinuria (PNH) is defined by acquired intravascular hemolytic anemia, thrombosis and bone marrow failure with pancytopenia. Systemic lupus erythematosus (SLE) also appears as an autoimmune disease. The coexistence of both is rarely reported. Here we report the case of a 30-year-old female presenting with pancytopenia and diagnosed as SLE, who also had a PNH clone. Bone marrow biopsy did not support hypoplastic anemia. The patient was then followed up with the consideration of the existence of a PNH clone with SLE. She was treated by the rheumatology department and complete blood count improved under immunosuppressive treatment. The coexistence of CD59-CD55 deficiency with autoimmune diseases has been reported. It is an important example in terms of receiving clinical response with SLE-specific treatment.
阵发性睡眠性血红蛋白尿（PNH）的定义为获得性血管内溶血性贫血、血栓形成以及伴有全血细胞减少的骨髓衰竭。系统性红斑狼疮（SLE）同样表现为一种自身免疫性疾病。两者并存的情况鲜有报道。在此，我们报告一例30岁女性病例，该患者表现为全血细胞减少，诊断为SLE，同时也存在PNH克隆。骨髓活检不支持再生障碍性贫血。随后对该患者进行随访，考虑其存在伴有SLE的PNH克隆。她接受了风湿科治疗，在免疫抑制治疗下全血细胞计数有所改善。CD59 - CD55缺陷与自身免疫性疾病并存的情况已有报道。就SLE特异性治疗获得临床反应而言，这是一个重要的例子。
