Ashrafi Farzaneh, Darakhshandeh Ali, Nematolahy Pardis, Khosravi Alireza
Assistant Professor, Department of Internal Medicine, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Resident, Department of Internal Medicine, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
ARYA Atheroscler. 2014 Sep;10(5):276-9.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal syndrome (POEMS) is a rare paraneoplastic syndrome associated with plasma cell dyscrasia.
A 48-year-old man presented with a 1-year history of paresthesia and progressive weakness of extremities. Diagnosis of POEMS syndrome was made for him on the basis of clinical presentation, additional physical findings, typical sclerotic bone lesion, and bone marrow findings. In last admission, he explained episodes of dyspnea and chest pain that associated with frequent premature ventricular contraction in his electrocardiograph. Patient heart monitoring showed some episodes of complete heart block. Infra-His atrioventricular block in electro-physiologic study was detected. He had no history of ischemic heart disease. His cardiopulmonary findings on examination were normal. All results of cardiac biomarkers and serum electrolytes and repeated echocardiography were within normal range. Cong red staining of rectal fat pad biopsy was negative. After pacemaker insertion radiation of sclerotic bone, lesion started for him, but radiotherapy was ineffective, and he expired with respiratory failure. Complete heart block in POEMS syndrome has not been reported previously, and it is the first POEMS case with complete heart block.
Complete heart block is a cardiac manifestation of POEMS syndrome.
多神经病、器官肿大、内分泌病、单克隆蛋白血症(POEMS)综合征是一种与浆细胞发育异常相关的罕见副肿瘤综合征。
一名48岁男性,有1年肢体感觉异常和进行性无力病史。根据临床表现、其他体格检查结果、典型的硬化性骨病变及骨髓检查结果,确诊为POEMS综合征。上次入院时,他诉说有呼吸困难和胸痛发作,心电图显示频发室性早搏。患者心脏监测显示有几次完全性心脏传导阻滞。电生理研究检测到希氏束以下房室传导阻滞。他无缺血性心脏病病史。体格检查时其心肺检查结果正常。心脏生物标志物、血清电解质及多次超声心动图检查的所有结果均在正常范围内。直肠脂肪垫活检刚果红染色阴性。植入起搏器后对硬化性骨病变进行放疗,但放疗无效,患者因呼吸衰竭死亡。此前未见POEMS综合征伴完全性心脏传导阻滞的报道,这是首例POEMS综合征伴完全性心脏传导阻滞病例。
完全性心脏传导阻滞是POEMS综合征的一种心脏表现。