Ohta Takanori, Oda Naoki, Saito Keiko, Tamiya Sadafumi, Ueno Toshiyuki
Department of Internal Medicine, Kitakyushu Municipal Medical Center, Kitakyushu, JPN.
Department of Pathology, Kitakyushu Municipal Medical Center, Kitakyushu, JPN.
Cureus. 2020 Dec 19;12(12):e12175. doi: 10.7759/cureus.12175.
A 50-year-old Japanese man complaining of dry mouth and eyes, pale skin with cold irritation, and worsening epigastric pain was admitted to the hospital, whereupon he developed fever and anasarca. A computed tomography (CT) scan showed ascites, hepatosplenomegaly, and mildly enlarged multiple lymph nodes, and blood examination revealed renal impairment, thrombocytopenia, and high levels of C-reactive protein (CRP). He was diagnosed with Sjögren syndrome and concurrently manifested symptoms resembling TAFRO syndrome (i.e., thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O)). Although the TAFRO syndrome-like symptoms progressed, he gradually recovered with immunosuppressive agents. Seven years and five months after the admission, the TAFRO syndrome-like symptoms recurred. Bone marrow biopsy specimens revealed reticulin fibrosis. Inguinal and mediastinal lymph nodes biopsy specimens revealed Castleman disease-like features. Although the symptoms indicated TAFRO syndrome, a diagnosis was not possible owing to the presence of hypergammaglobulinemia and Sjögren syndrome, which required exclusion. Corticosteroid treatment was initiated; however, it was complicated by retroperitoneal hemorrhage, probably due to microangiopathy. After additional treatment with tocilizumab and rituximab, the TAFRO syndrome-like symptoms improved and the hemorrhage progression stopped. In conclusion, TAFRO syndrome-like symptoms may recur with vascular complications and can be successfully treated with tocilizumab and rituximab during Sjögren syndrome. The etiology of TAFRO syndrome could potentially involve Sjögren syndrome, and these syndromes may co-exist.
一名50岁的日本男性因口干、眼干、遇冷刺激皮肤苍白以及上腹部疼痛加重而入院，随后出现发热和全身性水肿。计算机断层扫描（CT）显示有腹水、肝脾肿大以及多个淋巴结轻度肿大，血液检查显示肾功能损害、血小板减少和C反应蛋白（CRP）水平升高。他被诊断为干燥综合征，同时表现出类似TAFRO综合征的症状（即血小板减少（T）、全身性水肿（A）、发热（F）、网状纤维增生（R）和器官肿大（O））。尽管类似TAFRO综合征的症状有所进展，但他在使用免疫抑制剂后逐渐康复。入院7年零5个月后，类似TAFRO综合征的症状复发。骨髓活检标本显示网状纤维增生。腹股沟和纵隔淋巴结活检标本显示有Castleman病样特征。尽管症状提示为TAFRO综合征，但由于存在高球蛋白血症和干燥综合征需要排除，故无法确诊。开始使用皮质类固醇治疗；然而，出现了腹膜后出血并发症，可能是由于微血管病变所致。在使用托珠单抗和利妥昔单抗进行额外治疗后，类似TAFRO综合征的症状有所改善，出血进展停止。总之，类似TAFRO综合征的症状可能会伴有血管并发症复发，在干燥综合征期间使用托珠单抗和利妥昔单抗可成功治疗。TAFRO综合征的病因可能与干燥综合征有关，且这些综合征可能并存。
