Pasquesoone Henri, Callaud Aurélien, Carsuzaa Thibaut, Chalopin Thomas, Santiago-Ribeiro Maria-Joao
Nuclear Medicine Department, CHRU TOURS, Tours, France.
Hematology Department, CHRU Tours, Tours, France.
Front Nucl Med. 2023 Oct 26;3:1273967. doi: 10.3389/fnume.2023.1273967. eCollection 2023.
TEMPI syndrome (TEMPI) compounds telangiectasias and polycythemia with elevated erythropoietin levels, monoclonal gammopathy, perirenal fluid collections, and intrapulmonary shunt. Although the pathophysiology of this syndrome remains unclarified, prior research has been established that it is a plasma cell neoplasm, often containing less than 10% bone marrow plasma cells. F-FDG PET serves as a valuable instrument for initial staging and treatment monitoring in multiple myeloma management. Thus, F-FDG PET can be legitimately applied for TEMPI assessment. Here, we present the first F-FDG PET images for the initial evaluation and treatment monitoring of TEMPI in a 51-year-old woman, who exhibited polycythemia (EPO:5,448 mIU/ml) without JAK2 mutation, telangiectasias, monoclonal IgG lambda gammopathy (13.9) g/L and 7% dysmorphic plasma cells (CD38 + CD138+), occasionally clustered, in favor of tumoral plasmacytomas. The first PET scan exhibited hypermetabolic diffuse bone marrow, potentially related to polycythemia, accompanied by non-lytic bone hypermetabolic lesions in the femoral and humeral diaphysis, and ametabolic peri-renal fluid collections, brown fat, and pleural talcoma. Post-treatment F-FDG PET (Daratumumab Bortezomib Thalidomide Dexamethasone) revealed a completely reduced signal of bone lesions, suggesting a complete response, which was substantiated both clinically and biologically, with the concurrent disappearance of telangiectasia and the monoclonal component, and the normalization of the EPO level. In future, additional data will be required to confirm the added value of F-FDG PET with TEMPI. Nevertheless, F-FDG PET can be a preferred tool for the extension workup and therapeutic evaluation of TEMPI syndrome.
TEMPI综合征（TEMPI）合并毛细血管扩张症和红细胞增多症，伴有促红细胞生成素水平升高、单克隆丙种球蛋白病、肾周液体积聚和肺内分流。尽管该综合征的病理生理学仍未阐明，但先前的研究已证实它是一种浆细胞瘤，骨髓浆细胞通常少于10%。F-FDG PET是多发性骨髓瘤管理中初始分期和治疗监测的重要工具。因此，F-FDG PET可合理应用于TEMPI评估。在此，我们展示了首例F-FDG PET图像，用于对一名51岁女性TEMPI的初始评估和治疗监测，该女性表现为无JAK2突变的红细胞增多症（促红细胞生成素：5448 mIU/ml）、毛细血管扩张症、单克隆IgG λ丙种球蛋白病（13.9 g/L）以及7%形态异常的浆细胞（CD38 + CD138 +），偶尔聚集，提示肿瘤性浆细胞瘤。首次PET扫描显示骨髓弥漫性高代谢，可能与红细胞增多症有关，同时股骨和肱骨干有非溶骨性骨高代谢病变，以及无代谢的肾周液体积聚、棕色脂肪和胸膜滑石瘤。治疗后F-FDG PET（达雷妥尤单抗、硼替佐米、沙利度胺、地塞米松）显示骨病变信号完全降低，提示完全缓解，这在临床和生物学上均得到证实，同时毛细血管扩张症和单克隆成分消失，促红细胞生成素水平恢复正常。未来，需要更多数据来证实F-FDG PET对TEMPI的附加价值。尽管如此，F-FDG PET可以成为TEMPI综合征扩展检查和治疗评估的首选工具。
