TEMPI综合征于2011年首次定义,并于2016年归类为浆细胞肿瘤与相关副肿瘤综合征。该综合征的发病机制尚不清楚。识别毛细血管扩张的组合,红细胞增多与高红细胞生成素水平,单克隆丙种球蛋白病,肾周积液收集,肺内分流是控制疾病的第一步。诊断通常会延迟,因为该综合征很少见,可能会被误认为是其他皮肤病学,肾,和肺部疾病。如果没有早期诊断,肺损伤会导致严重的残疾。我们在这里发表的文章描述了一名58岁女性的TEMPI综合征的临床病例,这说明了及时识别这种不寻常疾病的困难。这里,我们还回顾了TEMPI综合征的临床特征,鉴别诊断和可用的治疗方案,基于当前的文献。虽然数量有限,随着新患者的加入,TEMPI综合征正在演变为特征良好的多系统综合征。这种罕见的疾病不应该错过,特别是如果患者被推定为患有单克隆丙种球蛋白病和脊髓灰质炎的原发性毛细血管扩张症。提高临床医生对该疾病的认识并在文献中增加新的患者数据可能有助于更好地理解该疾病的病理生理学和治疗的标准化。
TEMPI syndrome was first defined in 2011 and classified as a plasma cell neoplasm with associated paraneoplastic syndrome in 2016. The pathogenesis of the syndrome is not well understood. Recognition of a combination of
telangiectasia, erythrocytosis with a high erythropoietin level, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunt is the first step in managing the disease. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other dermatological, renal, and pulmonary disorders. Without early diagnosis significant disability results from the pulmonary damage. The article we present here describes a clinical
case of TEMPI-syndrome in a 58-year-old woman, which illustrates the difficulties associated with the timely recognition of this unusual disease. Here, we also review the clinical features of TEMPI syndrome, differential diagnosis and available treatment options, based on current literature. Although limited in number, with the addition of new patients to the literature, TEMPI syndrome is evolving into a well characterized multisystem syndrome. This rare disorder should not be missed, especially if the patient has a putative diagnosis of essential
telangiectasia with a monoclonal gammopathy and polistemia. Increasing the awareness of clinicians about the disease and adding new patient data to the literature may contribute to a better understanding of the pathophysiology of the disease and standardization of treatment.