Abstract：
OBJECTIVE: TEMPI (telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonaryshunting) syndrome is a rare multisystemic disease classified as a monoclonal gammopathy of cutaneous significance. The pathogenesis and etiology of TEMPIare not well known because of the rarity of this disorder. Although telangiectasias are the hallmark of this syndrome, skin biopsies are rarely performed. We aim to further characterize TEMPI syndrome through the evaluationof a skin biopsy.
METHODS: We reviewed the histopathology and immunophenotypic profile of a skin biopsy from a 53-year-oldwoman diagnosed with TEMPI syndrome. Other components of her syndromic complex included an IgA myeloma, elevated vascular endothelial growth factor (VEGF), and erythrocytosis.
RESULTS: A biopsy showed prominent vascular ectasia with some degree of microvascular basement membranezone thickening. Our patient had a reduction in neoplastic plasma cell burdenand clearing of her telangiectasias following myeloma directed treatment.
CONCLUSIONS: TEMPI can beviewed as a reactive vascular paraneoplastic syndrome in the setting of a plasma cell dyscrasia. Elaboration of VEGF from neoplastic plasma cells is likely pathogenetically implicated and appears to be a common link that explains other vascular lesions associated with monoclonal gammopathy syndromes.
METHODS: We reviewed the histopathology and immunophenotypic profile of a skin biopsy from a 53-year-oldwoman diagnosed with TEMPI syndrome. Other components of her syndromic complex included an IgA myeloma, elevated vascular endothelial growth factor (VEGF), and erythrocytosis.
RESULTS: A biopsy showed prominent vascular ectasia with some degree of microvascular basement membranezone thickening. Our patient had a reduction in neoplastic plasma cell burdenand clearing of her telangiectasias following myeloma directed treatment.
CONCLUSIONS: TEMPI can beviewed as a reactive vascular paraneoplastic syndrome in the setting of a plasma cell dyscrasia. Elaboration of VEGF from neoplastic plasma cells is likely pathogenetically implicated and appears to be a common link that explains other vascular lesions associated with monoclonal gammopathy syndromes.
摘要：
目标：TEMPI(毛细血管扩张，促红细胞生成素升高和红细胞增多，单克隆丙种球蛋白病，肾周积液集合,和肺内分流）综合征是一种罕见的多系统疾病，被归类为具有皮肤意义的单克隆丙种球蛋白病。由于这种疾病的罕见性，TEMPIs的发病机制和病因尚不为人所知。尽管毛细血管扩张是这种综合征的标志，很少进行皮肤活检。我们旨在通过皮肤活检的评估进一步表征TEMPI综合征。
方法：我们回顾了一名诊断为TEMPI综合征的53岁女性皮肤活检的组织病理学和免疫表型。她的综合征复合体的其他组成部分包括IgA骨髓瘤，血管内皮生长因子（VEGF）升高，和红细胞增多症。
结果：活检显示明显的血管扩张伴一定程度的微血管基底膜区增厚。骨髓瘤定向治疗后，我们的患者的肿瘤浆细胞负担减少，毛细血管扩张清除。
结论：TEMPI可被视为浆细胞异常背景下的反应性血管副肿瘤综合征。肿瘤浆细胞中VEGF的表达可能与发病机制有关，并且似乎是解释与单克隆丙种球蛋白综合征相关的其他血管病变的共同联系。
方法：我们回顾了一名诊断为TEMPI综合征的53岁女性皮肤活检的组织病理学和免疫表型。她的综合征复合体的其他组成部分包括IgA骨髓瘤，血管内皮生长因子（VEGF）升高，和红细胞增多症。
结果：活检显示明显的血管扩张伴一定程度的微血管基底膜区增厚。骨髓瘤定向治疗后，我们的患者的肿瘤浆细胞负担减少，毛细血管扩张清除。
结论：TEMPI可被视为浆细胞异常背景下的反应性血管副肿瘤综合征。肿瘤浆细胞中VEGF的表达可能与发病机制有关，并且似乎是解释与单克隆丙种球蛋白综合征相关的其他血管病变的共同联系。
