Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece.
1st Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
Ann Hematol. 2024 Sep;103(9):3787-3793. doi: 10.1007/s00277-024-05893-8. Epub 2024 Jul 30.
TEMPI syndrome is a rare, acquired disorder with multisystemic manifestations. It is classified as a plasma cell disorder and is characterized by telangiectasias, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunt. Even though TEMPI's pathophysiology remains elusive, it responds to anti-myeloma therapy indicating that the monoclonal protein or clone plays a key role. We present a challenging case of a 73-year-old man with erythrocytosis and deteriorating renal function with nephrotic-range proteinuria in whom after extensive work up, the diagnosis of TEMPI syndrome was made. He was received treatment with daratumumab-bortezomib-cyclophosphamide and dexamethasone (Dara-VCD) and achieved a hematological and clinical response. We also report preliminary data on a multiplex assay for cytokines and growth factors for two patients with TEMPI syndrome and note lower levels for non-specific innate immunity related cytokines. A direct link between renal impairment and TEMPI syndrome is not currently established; cytokine deregulation could potentially be involved in the ischemic changes observed in the renal biopsy of our patient.
TEMPI 综合征是一种罕见的获得性多系统疾病。它被归类为浆细胞疾病，其特征是毛细血管扩张、红细胞增多、单克隆丙种球蛋白病、肾周积液和肺内分流。尽管 TEMPI 的病理生理学仍然难以捉摸，但它对骨髓瘤治疗有反应，表明单克隆蛋白或克隆起着关键作用。我们报告了一例 73 岁男性的挑战性病例，该患者患有红细胞增多症和进行性肾功能衰竭，伴有肾病范围蛋白尿，经过广泛检查，诊断为 TEMPI 综合征。他接受了达雷妥尤单抗-硼替佐米-环磷酰胺和地塞米松（Dara-VCD）治疗，并获得了血液学和临床反应。我们还报告了两名 TEMPI 综合征患者的细胞因子和生长因子的多重分析的初步数据，并注意到非特异性固有免疫相关细胞因子的水平较低。肾脏损害与 TEMPI 综合征之间目前没有直接联系；细胞因子失调可能与我们患者的肾活检中观察到的缺血性改变有关。
