Hayashino Kenta, Fujii Nobuharu, Nagano Tomohiro, Ikeda Daisuke, Fujiwara Kanako, Hashida Risa, Kitamura Wataru, Kobayashi Hiroki, Kondo Takumi, Seike Keisuke, Fujiwara Hideaki, Asada Noboru, Ennishi Daisuke, Fujii Keiko, Matsuoka Ken-Ichi, Maeda Yoshinobu
Department of Hematology and Oncology, Okayama University Hospital, 2-5-1 Shikata, Okayama, Japan.
Division of Transfusion and Cell Therapy, Okayama University Hospital, 2-5-1 Shikata, Okayama, Japan.
Int J Hematol. 2025 May;121(5):706-711. doi: 10.1007/s12185-025-03937-x. Epub 2025 Feb 4.
TAFRO syndrome is a systemic disease characterized by thrombocytopenia, anasarca, fever, systemic inflammation, reticulin fibrosis, renal insufficiency, and organomegaly. Although the pathogenesis of TAFRO syndrome remains unknown, it may be associated with cytokine storm and abnormal immune function. Herein, we present a case of a 65-year-old man who was diagnosed with myelodysplastic neoplasms (MDS) with repeating TAFRO syndrome-like symptoms. At ages 59 and 63 years, he developed TAFRO syndrome and was treated with immunosuppressive therapy, which improved these symptoms. At age 65 years, he had TAFRO syndrome-like symptoms with pancytopenia, chromosomal abnormalities, and dysplasia. The patient was subsequently diagnosed with MDS and treated with methylprednisolone, rituximab, bortezomib, and tocilizumab. His MDS-related and TAFRO syndrome-like symptoms simultaneously improved following treatment. Although the patient was not diagnosed with MDS at the first and second events, chromosomal abnormalities were detected, revealing increased clonal cells. MDS can be complicated by immune disorders associated with increased malignant clonal cells. Additionally, patients with MDS exhibit hypercytokinemia, including interleukin-6 and vascular endothelial growth factor. This case indicates that increased clonal cells and hypercytokinemia caused by MDS may lead to abnormal immune function and induce TAFRO syndrome-like symptoms.
TAFRO综合征是一种全身性疾病，其特征为血小板减少、全身性水肿、发热、全身炎症、网状纤维增生、肾功能不全和器官肿大。尽管TAFRO综合征的发病机制尚不清楚，但可能与细胞因子风暴和免疫功能异常有关。在此，我们报告一例65岁男性患者，该患者被诊断为骨髓增生异常肿瘤（MDS），伴有反复出现的TAFRO综合征样症状。在59岁和63岁时，他患上了TAFRO综合征，并接受了免疫抑制治疗，这些症状得到了改善。65岁时，他出现了TAFRO综合征样症状，伴有全血细胞减少、染色体异常和发育异常。该患者随后被诊断为MDS，并接受了甲泼尼龙、利妥昔单抗、硼替佐米和托珠单抗治疗。治疗后，他与MDS相关的症状和TAFRO综合征样症状同时得到改善。尽管该患者在第一次和第二次发病时未被诊断为MDS，但检测到了染色体异常，提示克隆细胞增多。MDS可并发与恶性克隆细胞增多相关的免疫紊乱。此外，MDS患者表现出高细胞因子血症，包括白细胞介素-6和血管内皮生长因子。该病例表明，MDS导致的克隆细胞增多和高细胞因子血症可能导致免疫功能异常，并诱发TAFRO综合征样症状。
