Nakamura Norio, Sugawara Toshiyuki, Shirato Ken-Ichi, Kumasaka Ryuichiro, Nakamura Masayuki, Shimada Michiko, Fujita Takeshi, Murakami Reiichi, Shimaya Yuko, Osawa Hiroshi, Yamabe Hideaki, Okumura Ken
Community Medicine, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki-city, Aomori, 036-8562, Japan.
J Med Case Rep. 2011 Nov 14;5:550. doi: 10.1186/1752-1947-5-550.
Paroxysmal nocturnal hemoglobinuria is an acquired disorder of hemopoiesis and is characterized by recurrent episodes of intravascular hemolysis due to an increased sensitivity to complement-mediated hemolysis. Systemic lupus erythematosus with paroxysmal nocturnal hemoglobinuria is very rare. We report a case of paroxysmal nocturnal hemoglobinuria that developed in a patient with systemic lupus erythematosus and lupus nephritis.
A 29-year-old Mongolian woman had systemic lupus erythematosus, which manifested only as skin lesions when she was 12 years old. She had leg edema and proteinuria when she was 23 years old, and a renal biopsy revealed lupus nephritis (World Health Organization type IV). She had been treated with steroids and immunosuppressant therapy. At 29, she had headaches, nausea, general fatigue, and severe pancytopenia and was admitted to our hospital. A laboratory evaluation showed hemolytic anemia. Further examination showed a neutrophil alkaline phosphatase score of 46 points, a CD55 value of 18%, and a CD59 value of 78.6%. The results of Ham test and sugar water tests were positive. The constellation of symptoms throughout the clinical course and the laboratory findings suggested paroxysmal nocturnal hemoglobinuria.
To the best of our knowledge, systemic lupus erythematosus with paroxysmal nocturnal hemoglobinuria is very rare. Clinicians should be aware of the association between autoimmune and hematological diseases.
阵发性睡眠性血红蛋白尿是一种获得性造血系统疾病，其特征是由于对补体介导的溶血敏感性增加而反复发生血管内溶血。系统性红斑狼疮合并阵发性睡眠性血红蛋白尿非常罕见。我们报告一例系统性红斑狼疮和狼疮性肾炎患者发生阵发性睡眠性血红蛋白尿的病例。
一名29岁的蒙古族女性患有系统性红斑狼疮，12岁时仅表现为皮肤病变。23岁时出现腿部水肿和蛋白尿，肾活检显示为狼疮性肾炎（世界卫生组织IV型）。她接受了类固醇和免疫抑制治疗。29岁时，她出现头痛、恶心、全身乏力和严重全血细胞减少症，被收入我院。实验室检查显示溶血性贫血。进一步检查显示中性粒细胞碱性磷酸酶评分为46分，CD55值为18%，CD59值为78.6%。酸溶血试验和糖水试验结果均为阳性。整个临床过程中的一系列症状和实验室检查结果提示阵发性睡眠性血红蛋白尿。
据我们所知，系统性红斑狼疮合并阵发性睡眠性血红蛋白尿非常罕见。临床医生应意识到自身免疫性疾病和血液系统疾病之间的关联。
