回顾性分析2019 年11 月14 日我科收住的l例IgG4相关性肺疾病合并甲状腺受累患者的临床资料、诊治及随访经过,并检索国内外文献,总结归纳.提高临床医师对IgG4 相关性肺疾病合并甲状腺受累的认识水平.

SLE是一种自身免疫病,可累及多个系统,既有免疫性血小板减少,又有自身免疫性溶血性贫血者,称为 Evans 综合征(Evans syndrome,ES).Schnitzler 综合征(Schnitzler syndrome,SchS)是一种罕见的与M蛋白血症相关的获得性自身炎症性疾病,以单克隆免疫球蛋白(M蛋白)和慢性荨麻疹为特征.目前,针对SchS的皮疹,主要治疗药物是IL-1拮抗剂,对于M蛋白,尚无较好的治疗方法.当SLE患者继发了 ES,同时又合并SchS时,治疗更加困难.本文报告1例SLE ES并发SchS患者,先予贝利尤单抗治疗,但M蛋白控制欠佳,这一状况在换为泰它西普后得到改善.

目的:观察DNA免疫吸附治疗对系统性红斑狼疮(SLE)患者抗dsDNA抗体的清除作用以及序贯联合免疫抑制剂治疗的疗效反应.方法:收集2017年8月至2021年8月荆州市中心医院风湿免疫科收治的SLE患者,选择体内抗dsDNA抗体高滴度阳性的活动期SLE患者36例,常规治疗基础上联合DNA免疫吸附治疗,观察患者治疗前、后自身抗体及血液生化指标的变化,吸附治疗后给予免疫抑制剂,随访至少12周.结果:吸附治疗后患者体内抗dsDNA抗体滴度显著下降,平均降幅(66.4％±12.8％),高滴度抗核抗体的患者显著减少.随访中,33例患者病情维持稳定,3例患者在1～2年后,抗dsDNA抗体再次上升,重复行DNA免疫吸附治疗,病情稳定.结论:DNA免疫吸附可以在短期内明显清除抗dsDNA抗体,有助于快速缓解病情,序贯联合免疫抑制剂治疗有助于实现疾病的长期稳定.

Felty’s syndrome (FS) is a deforming disease, characterized by the triad of rheumatoid arthritis (RA), neutropenia, and splenomegaly. Currently, FS patients are treated mainly with immunosuppressants, such as methotrexate and glucocorticoids, which however are not suitable to some patients and may cause severe side effects. Here we report a clinical FS case that was treated with Tocilizumab (TCZ) successfully. The patient had symmetrical swelling and pain of multiple joints, deformity of elbow joints with obvious morning stiffness. Joint color Doppler ultrasound showed synovial hyperplasia and bone erosion of wrist and proximal interphalangeal joints and CT scan suggested splenomegaly. Further examination showed neutropenia and anemia, a high titer of anti-cyclic citrullinated peptide antibody, rheumatoid factor and anti-nuclear antibodies, positive p-ANCA, and elevated IgA and IgG. After treating with TCZ, the patient has been relieved of clinical symptoms. His spleen has recovered to normal size. The absolute neutrophil count (ANC) tended to be stable, and joint erosion did not deteriorate. We have reviewed the literatures on FS treatment with biological agents and found only a few reports using TNF-α antagonist and rituximab treating FS, but none with TCZ. So, it is the first time to report a successful FS case treated with TCZ. This case suggests that the TCZ may be a new choice for FS treatment, under the condition of closely monitoring the ANC.

目的评价国产猪抗淋巴细胞球蛋白+环孢素治疗重型再生障碍性贫血的疗效。方法回顾性分析36例重型再生障碍性贫血应用猪抗淋巴细胞球蛋白联合环孢素治疗临床资料,观察治疗疗效、并发症及转归,并根据患者的年龄、性别、病程长短、重型再生障碍性贫血类型、网织红细胞计数、治疗前中性粒细胞计数、对粒细胞集落刺激因子的治疗有无反应以及免疫抑制治疗后前2周环孢素血药浓度进行分层分析。结果随访的36例中基本治愈9例(25%),达到缓解7例(19.44%),明显进步11例(30.56%),总有效率75%。单项资料对比分析显示,治疗有效率与患者年龄、性别、病程长短、重型再生障碍性贫血类型、外周血网织红细胞计数等无明显相关性,但治疗前外周血中性粒细胞计数≥0.2×109/L、对粒细胞集落刺激因子的治疗有反应以及免疫抑制治疗后前2周环孢素血药浓度≥200 ng/m l患者有效率明显增高。结论国产猪抗淋巴细胞球蛋白联合环孢素治疗重型再生障碍性贫血总体疗效好,相对安全。治疗前患者外周血中性粒细胞计数、对粒细胞集落刺激因子的治疗反应以及免疫抑制治疗后前两周环孢素血药浓度可能对疗效判断有提示意义。

目的:观察硼替佐米联合地塞米松治疗多发性骨髓瘤的临床疗效、不良反应。方法:结合文献报道,回顾性分析我院5例硼替佐米联合地塞米松治疗多发性骨髓瘤临床资料。结果:5例患者中,1例完全缓解(CR),2例非常好的部分缓解(VGPR),1例部分缓解(PR),1例轻微治疗反应(MR),总反应率为80%;主要不良反应有胃肠道症状、周围神经病、不同程度的白细胞减少、血小板减少、带状疱疹、侵袭性肺曲霉病,经过对症治疗后均能改善。结论:硼替佐米联合地塞米松治疗多发性骨髓瘤是一种安全、可靠、有较好治疗前景的方法。

Objective:To investigate the efficacy and safety of lower dose rituximab in the treatment of patients with refractory idiopathic thrombocytopenic purpura(ITP).Methonds: Four patients with ITP admitted to our hospital were given intravenous rituximab.Their clinical data were analyzed retrospective.Results: A complete response(platelet count greater than 100×109/L) was observed in 1 case,a response(platelet count greater than 30 ×109/L or with a 2-fold or higher increase of the inclusion value without any signs of bleeding) in 2 cases,with an overall response rate of 75%.No infusion related or delayed toxicities attributable to rituximab were experienced by any of the patients.Conclusions: Lower dose rituximab is beneficial for patients with ITP.Lower dose rituximab seems to show similar activity to standard dose.Its side effects are predictable and manageable.

目的:探讨多发性骨髓瘤的临床特点、误诊原因,降低误诊率。方法:回顾性分析2006年1月至2009年8月多发性骨髓瘤30例患者临床资料、误诊情况。结果:①误诊病种多样,常见误诊疾病涉及骨骼、呼吸、肾脏、心血管、血液等多系统疾病;②多发性骨髓瘤临床表现多样化,症状缺乏特异性;③多数医生对多发性骨髓瘤复杂多样的临床表现缺乏系统思考,局限于专科问题分析。结论:提高对多发性骨髓瘤的认识,抓住要点、及时进行相关检查,才能降低多发性骨髓瘤的误诊率。

例1,男,19岁,因"急性早幼粒细胞白血病"复发给予维甲酸40mg/d治疗9天,其后予DA方案化疗3天,于化疗后第3天因高热,咳嗽,胸闷,气促,视物不清入住我院.查体:重度贫血貌,颜面浮肿,注射部位片状瘀斑,双肺呼吸音减低,HR 105次/分.