Nishina M, Saito E, Kinoshita M
Fourth Department of Internal Medicine, School of Medicine, Toho University, Tokyo.
Ryumachi. 1996 Oct;36(5):757-61.
A 47 year old woman was presented in June, 1994 with a ten years history of systemic lupus erythematosus. Since six months before first visit to our hospital, she had experienced general malaise, myalgia and persistent low grade fever. Among this period, titers of anti DNA antibody in her blood were gradually increased. She had been treated with the prednisolone which was step-wisely increased up to 15 mg/day, but her symptoms had been worsened. On admission, physical examination showed low grade fever and erythema on her both hands. Investigations showed her white blood cell count 1400/microliter, platelet 90000/microliter, positive test result for ANA, anti DNA antibody and anti Sm antibody, low serum concentrations of complements, and persistent proteinuria. She was treated with 60 mg/day of oral prednisolone. Though her symptoms and immunological abnormalities were gradually improved, her platelet count and white blood cell count were gradually decreased to 50000/microliter and 1400/microliter in August, respectively. Platelet associated IgG was increased (102.9 ng/10(7) cells). In spite of the various trials of treatment, such as plasmapheresis, high-dose intravenous gammaglobulin, and cyclophosphamide, the white blood cell and platelet number could not be raised. Finally, the administration of 75 mg/day of dapsone brought a significant rise in both blood cell counts. Thus being certainly effective against leukocytopenia and thrombocytopenia, dapsone should be considered an one of the beneficial therapeutic procedures for refractory autoimmune leukocytopenia and thrombocytopenia in SLE patients.
一名47岁女性于1994年6月就诊,有10年系统性红斑狼疮病史。自首次来我院就诊前6个月起,她出现全身不适、肌痛和持续低热。在此期间,她血液中的抗DNA抗体滴度逐渐升高。她曾接受泼尼松龙治疗,剂量逐步增加至15毫克/天,但症状仍加重。入院时,体格检查发现低热,双手有红斑。检查显示她的白细胞计数为1400/微升,血小板为90000/微升,抗核抗体、抗DNA抗体和抗Sm抗体检测结果呈阳性,血清补体浓度低,且持续蛋白尿。她接受了60毫克/天的口服泼尼松龙治疗。尽管她的症状和免疫异常逐渐改善,但她的血小板计数和白细胞计数在8月分别逐渐降至50000/微升和1400/微升。血小板相关IgG升高(102.9纳克/10⁷细胞)。尽管进行了各种治疗尝试,如血浆置换、大剂量静脉注射丙种球蛋白和环磷酰胺,但白细胞和血小板数量仍无法升高。最后,给予75毫克/天的氨苯砜使血细胞计数显著上升。因此,氨苯砜对白细胞减少和血小板减少肯定有效,应被视为系统性红斑狼疮患者难治性自身免疫性白细胞减少和血小板减少的有益治疗方法之一。