POEMS综合征患者就诊行为特征及临床误诊漏诊分析

Analysis of misdiagnosis and missed diagnosis of POEMS with respect to hospital visit patterns

目的:了解POEMS综合征患者就诊行为特征,分析POEMS综合征临床误诊、漏诊原因。方法:回顾性分析在中国医学科学院血液病医院就诊的30例POEMS综合征患者的临床资料,包括患者初诊年龄、性别、首发症状及就诊过程等。结果:30例POEMS综合征患者的中位发病年龄为51(26~67)岁,男女之比为1.1∶1,自发病至明确诊断期间的中位就诊次数和时间分别为3(1~6)次和13(1.5~132)个月。患者就诊科室多为神经内科、肾内科、内分泌科、消化科,但确诊科室集中在血液内科。患者临床误诊、漏诊现象普遍存在,误诊及漏诊率高达87%。常见的首诊诊断包括格林巴利综合征、周围神经病和诊断不明。近一半患者在此后多次就诊的过程中更改了原有诊断,部分患者曾疑诊POEMS综合征但均未得到进一步证实。确诊前患者接受的治疗方式包括对症支持、丙种球蛋白+糖皮质激素冲击治疗、糖皮质激素治疗和观察随访。73%(19/26)的患者在治疗过程中出现病情加重。误诊及漏诊的主要原因为POEMS综合征临床表现多样且非特异,患者和医务工作者不易识别;此外,接诊医师专注于本学科范围相关的症状体征,忽略了专科之外的临床表现,也是导致误诊及漏诊的主要原因。结论:由于POEMS综合征较为罕见且临床表现高度异质性,临床误诊、漏诊较为常见。若接诊医师能够从“一元论”角度探究病因,将本学科范围内症状体征与专科外临床表现联系在一起,加强多学科合作,可有效减少漏诊和误诊的发生。

Objective:To analyze clinical visit patterns and the reasons pertaining to misdiagnosis and missed diagnosis of POEMS syndrome.Methods:The clinical data,including age,sex,symptoms,and process of arriving at the initial diagnosis,of 30 patients with POEMS syndrome in our hospital were analyzed retrospectively.Results:The median patient age was 51(26−67)years,and the ratio of males to females was 1.1:1.The median number of visits and median time from onset to definite diagnosis were 3(1−6)times and 13(1.5−13.2)months,respectively.Though the departments of neurology,nephrology,endocrinology,and digestion were initially visited by the patients,the department with the most diagnoses was hematology.Twenty-six(87%)patients experienced misdiagnosis or missed diagnosis.The common initial diagnoses included Guillain-Barrésyndrome,peripheral neuropathy,and unknown diagnosis.Additionally,nearly half of the original diagnoses were changed during the patients’visiting process since then,wherein only some patients were suggested of having POEMS syndrome,but none of them were further examined.The treatment regimen administered before a confirmed diagnosis included supportive therapy,intravenous immunoglobulin combined with glucocorticoid implosive therapy,glucocorticoid therapy,and observation;73%(19/26)of patients experienced exacerbation of their condition during treatment.Furthermore,the clinical manifestations of POEMS syndrome were highly heterogeneous and not easily recognized by the consulting physicians.Clinicians tend to focus on symptoms and signs related to their specialty,ignoring clinical manifestations beyond the scope of their specialty-this is the main reason for misdiagnosis and missed diagnosis.Conclusions:Misdiagnosis and missed diagnosis are common in POEMS syndrome owing to its rarity and clinical heterogeneity.These lapses can be effectively reduced if clinicians associate clinical manifestations both with their respective field and other fields,thereby strengthening multidisciplinary cooperation.