Park Y H, Sunamoto M, Miyoshi T, Konaka Y
Department of Internal Medicine, Kitano Hospital.
Rinsho Ketsueki. 1993 Jul;34(7):870-5.
A 57-year-old man was admitted with massive nasal bleeding and blurred vision in January, 1991. Laboratory examination showed a prominent decrease of platelet number (1,000/microliters) and a marked elevation of PAIgG (4,025 ng/10(7) cells). Serological test revealed positive antinuclear factor, low concentration of C3 and C4, high level of immune complex and polyclonal hypergammaglobulinemia. The patient had uveitis and bilateral hilar lymphadenopathy with a high level of serum lysozyme and negative PPD skin test. The diagnosis of SLE complicated with thrombocytopenia and sarcoidosis was made. In spite of the various trials of treatment, such as oral prednisolone (PSL), methyl-PSL pulse therapy, plasma exchange, high-dose intravenous gammaglobulin, cyclophosphamide, azathioprine, vincristine, colchicine, cyclosporine-A, mizoribine, danazol, ascorbic acid and interferon alpha 2b, the platelet number could not be raised enough to keep more than 10,000/microliters, though the level of PAIgG decreased to 200 ng/10(7) cells. Finally, the administration of 75 mg/day of dapsone brought about a significant rise in platelet number within 2 weeks. The maximum number of 6.2 x 10(4)/microliters was obtained after 2 months. Then the patient stopped himself to take the drug, but the platelet number remained around 4-5 x 10(4)/microliters. Same dose of the drug was again prescribed to confirm the effect of dapsone. The platelet number increased to 7.9 x 10(4)/microliters in 2 weeks, and gradually returned to 5 x 10(4)/microliters after cessation of the drug. Thus being certainly effective against thrombocytopenia, dapsone should be considered as one of the therapeutic choice for refractory autoimmune thrombocytopenia.
一名57岁男性于1991年1月因大量鼻出血和视力模糊入院。实验室检查显示血小板数量显著减少（1000/微升），PAIgG明显升高（4025 ng/10⁷细胞）。血清学检查显示抗核因子阳性、C3和C4浓度降低、免疫复合物水平升高以及多克隆高球蛋白血症。患者患有葡萄膜炎和双侧肺门淋巴结病，血清溶菌酶水平升高，PPD皮肤试验阴性。诊断为系统性红斑狼疮合并血小板减少症和结节病。尽管进行了各种治疗尝试，如口服泼尼松龙（PSL）、甲泼尼龙脉冲疗法、血浆置换、大剂量静脉注射丙种球蛋白、环磷酰胺、硫唑嘌呤、长春新碱、秋水仙碱、环孢素A、咪唑立宾、达那唑、维生素C和干扰素α2b，但血小板数量仍无法升至足以维持在10000/微升以上，尽管PAIgG水平降至200 ng/10⁷细胞。最后，每天服用75毫克氨苯砜在2周内使血小板数量显著上升。2个月后达到最高值6.2×10⁴/微升。然后患者自行停药，但血小板数量仍维持在4 - 5×10⁴/微升左右。再次给予相同剂量的药物以确认氨苯砜的效果。血小板数量在2周内升至7.9×10⁴/微升，停药后逐渐恢复至5×10⁴/微升。因此，氨苯砜对血小板减少症肯定有效，应被视为难治性自身免疫性血小板减少症的治疗选择之一。
