高γ球蛋白血症性紫癜10例临床分析

Clinical analysis of 10 cases of hypergamaglobulinaemica purpura

目的:分析高γ球蛋白血症性紫癜(HGP)的临床表现、实验室检查特点,并复习相关文献,为该病的正确诊治提供临床依据。方法:收集于2008年9月—2018年9月华中科技大学同济医学院附属协和医院皮肤科收治住院的10例HGP患者的临床资料进行回顾性分析。结果:10例HGP患者中,男性3例,女性7例;发病年龄25~70岁,平均(48.10±13.99)岁。病程2个月~10余年,其中3例为初发(30%),7例为复发(70%)。皮损均为针尖至绿豆大淤点,压之不褪色,有褐色色沉。皮损位于四肢2例(20%),位于双下肢8例(80%)。6例入院诊断为过敏性紫癜,误诊率为60%;4例合并干燥综合征,1例合并慢性阻塞性肺疾病。所有病例蛋白电泳检查均提示γ球蛋白高于正常值。所有患者均采用葡萄糖酸钙注射液20 mL+维生素C注射液2 g及丹参川芎注射液20 mL静脉滴注;复方甘草酸苷50 mg,每日3次,口服;外用多磺酸黏多糖软膏。伴瘙痒者加用抗组胺药物,伴有干燥综合征者加用白芍总苷0.6 g,每日3次,口服;人工泪液滴眼,每日3次。有1例合并干燥综合征,且干燥性症状明显者,加用小剂量糖皮质激素治疗。所有患者症状均有不同程度改善。结论:HGP临床较少见,易误诊,常合并自身免疫性疾病,应进行系统检查。

Objective: To investigate the clinical and experimental features of hypergamaglobulinaemica purpura. Methods:The clinical data of 10 cases with hypergamaglobulinaemica purpura confirmed based on the clinical manifestations and laboratory examination were retrospectively analyzed. Results: There were 3 males and 7 females. The onset age ranged from 25 to70 years with a mean age of(48.10±13.99) years. The duration ranged from 2 months to more than 10 years. Three cases were diagnosed for the first time(30%) and seven were recurrent cases(70%). The skin lesions were pinhead-to soybean-sized petechiae mainly located on the extremities(2 cases, 20%) and legs(8 cases, 80%). Six cases were misdiagnosed as allergic purpura. The misdiagnosis rate was 60%. Four cases were complicated with Sj?gren′s syndrome, and one with chronic obstructive pulmonary disease. All the patients showed increased gama-globulin. All patients received intravenous injection of calcium gluconate(20 ml), vitamin C(2 g) and salvia miltiorrhiza chuanxiong(20 mL). Compound glycyrrhizin(50 mg) were administered three times a day orally and MPS ointment was given for external use. Antihistamines were added for patients with pruritus. Total glucosides of paeony(oral, 0.6 g) and artificial tears were given for patients with Sj?gren′s syndrome, three times a day. The patient with Sj?gren′s syndrome, had obvious symptoms of xerosis and therefore was treated with low-dose glucocorticoid. The patients′ symptoms were improved to varying degrees. Conclusion: Hypergamaglobulinaemica purpura is rare and commonly complicated with autoimmune diseases. It can easily be misdiagnosed. A systemic examination is warranted for the disease.