Zhang Xianrui, Fang Meiyun
Department of Hematology, First Affiliated Hospital, Dalian Medical University, Dalian, People's Republic of China.
Department of Hematology, Affiliated ZhongShan Hospital of Dalian Medical University, Dalian, People's Republic of China.
Clin Lymphoma Myeloma Leuk. 2018 Nov;18(11):724-730. doi: 10.1016/j.clml.2018.07.284. Epub 2018 Aug 9.
TEMPI (telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting) syndrome is a newly described clinical entity that is generally considered a plasma cell dyscrasia with multiple system involvement. The etiology and pathophysiology of this condition remains elusive. Nevertheless, clonal plasma cells and monoclonal protein appear to be major contributors. The early diagnosis of TEMPI syndrome is essential because therapies targeting the underlying plasma cells can lead to a dramatic response. Bortezomib-based chemotherapy, daratumumab monotherapy, and autologous hematopoietic stem cell transplantation can result in reversal of most manifestations. Nevertheless, the diagnosis of TEMPI syndrome remains a substantial challenge owing to its rarity and the complexity of clinical presentations. TEMPI syndrome is often misdiagnosed as other causes of erythrocytosis, resulting in a delayed diagnosis and further clinical deterioration. The aim of the present review was to present the clinical and biologic features of TEMPI syndrome, highlighting the differential diagnosis and outlining the present understanding of its pathophysiology and treatment.
TEMPI（毛细血管扩张、促红细胞生成素升高伴红细胞增多症、单克隆丙种球蛋白病、肾周积液、肺内分流）综合征是一种新描述的临床实体，通常被认为是一种累及多个系统的浆细胞异常增殖性疾病。该病症的病因和病理生理学仍不清楚。然而，克隆性浆细胞和单克隆蛋白似乎是主要因素。TEMPI综合征的早期诊断至关重要，因为针对潜在浆细胞的治疗可产生显著疗效。基于硼替佐米的化疗、达雷妥尤单抗单药治疗以及自体造血干细胞移植可使大多数症状得到缓解。然而，由于其罕见性和临床表现的复杂性，TEMPI综合征的诊断仍然是一项重大挑战。TEMPI综合征常被误诊为其他导致红细胞增多症的病因，从而导致诊断延迟和病情进一步恶化。本综述的目的是介绍TEMPI综合征的临床和生物学特征，强调鉴别诊断，并概述目前对其病理生理学和治疗的认识。
