以难治性下消化道术后出血伴凝血功能障碍为特征的未确定意义单克隆γ病(MGUS)。

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL Intractable & rare diseases research Pub Date : 2022-11-01 DOI:10.5582/irdr.2022.01120
Shuxia Zhang, Zhifen Chen, Meijuan Huang, Rong Zhan, Long Wu, Qian Zhang, Yu Lin, Weizhong Jiang, Pan Chi, Feng'e Yang
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引用次数: 0

摘要

出血是下消化道手术后常见的并发症,但因凝血功能障碍引起的出血病例并不多见。本文作者遇到一位54岁的中国男性,经内镜直肠息肉切除术后出血难治性,多次内镜和手术干预均未能控制出血。APTT混合试验不能纠正,也没有自身免疫相关疾病的证据,因此考虑存在非特异性抗体。经经验性环磷酰胺和糖皮质激素治疗后,APTT得到纠正,胃肠道出血停止。根据实验室结果和治疗结果,患者最终被诊断为由未确定意义的单克隆γ病(MGUS)诱导的延长APTT。以APTT延长为特征的MGUS和凝血功能障碍很少有报道。本文回顾了单克隆免疫球蛋白升高和凝血功能障碍的研究。如果延长的APTT不确定的意义,不能纠正与APTT混合试验,如果自身免疫相关因素被排除,则需要考虑浆细胞相关疾病,如MGUS。
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Monoclonal gammopathy of undetermined significance (MGUS) characterized by refractory lower gastrointestinal postoperative bleeding with coagulopathy.

Bleeding is a common complication after lower gastrointestinal surgery, and cases due to coagulation dysfunction are rare. The current authors encountered a 54-year-old Chinese man with refractory bleeding after endoscopic rectal polypectomy, and multiple endoscopic and surgical interventions failed to control that bleeding. An APTT mixing test could not be corrected and there was no evidence of autoimmune-related disease, so the presence of nonspecific antibodies was considered. After empiric therapy with a cyclophosphamide and glucocorticoid, APTT was corrected and gastrointestinal bleeding stopped. Based on laboratory results and therapeutic results, the patient was ultimately diagnosed with prolonged APTT induced by monoclonal gammopathy of undetermined significance (MGUS). MGUS and coagulopathy characterized by a prolonged APTT has rarely been reported. Here, studies noting elevated monoclonal immunoglobulins and coagulopathy have been reviewed. If a prolonged APTT of undetermined significance cannot be corrected with an APTT mixing test and if autoimmune-related factors are excluded, then plasma cell-related diseases such as MGUS need to be considered.

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来源期刊
Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
自引率
0.00%
发文量
29
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