Tominaga Ryutaro, Umino Kento, Honda Seina, Yokoyama Daizo, Noguchi Atsuto, Furuki Shuka, Koyama Shunsuke, Murahashi Rui, Nakashima Hirotomo, Hyodo Kazuki, Kawaguchi Shin-Ichiro, Toda Yumiko, Minakata Daisuke, Ashizawa Masahiro, Yamamoto Chihiro, Hatano Kaoru, Sato Kazuya, Ohmine Ken, Fujiwara Shin-Ichiro, Kanda Yoshinobu
Division of Hematology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.
Division of Cell Transplantation and Transfusion, Jichi Medical University, Tochigi, Japan.
Int J Hematol. 2025 May;121(5):658-669. doi: 10.1007/s12185-025-03933-1. Epub 2025 Jan 29.
The study aimed to investigate the therapeutic effect of various initial treatments incorporating glucocorticoid (GC) in TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly). Cases of TAFRO syndrome up to November 2023 were retrospectively collected. Overall survival (OS) and resistance to GC therapy were assessed, with resistance analyzed based on the time to the next treatment or death (TTNTD). The study included 95 patients, including 5 diagnosed at our hospital. OS did not differ significantly between patients who received GC monotherapy and those who had a second-line therapy added within 2 weeks (100-day OS rate: 86.6% vs. 77.7%; p = 0.338). Moreover, 100-day OS did not differ between patients who received GC pulse therapy within 2 weeks and those who did not (77.5% vs. 93.1%, p = 0.129). In multivariate analyses, pretreatment severity score ≥ 8 (hazard ratio [HR], 2.99; 95% confidence interval [CI] 1.05-8.50) and platelets ≥ 6.9 × 10^4/µL (HR, 2.26; 95% CI 1.01-5.02) were significantly associated with shorter TTNTD. Additional second-line or GC pulse therapy provided no advantage in the hyperacute phase. Higher severity scores and platelet values may predict resistance to GC therapy.
本研究旨在探讨在TAFRO综合征（血小板减少、全身性水肿、发热、网状纤维增生和器官肿大）中采用包含糖皮质激素（GC）的各种初始治疗方法的疗效。回顾性收集了截至2023年11月的TAFRO综合征病例。评估总生存期（OS）和对GC治疗的耐药性，并根据下次治疗或死亡时间（TTNTD）分析耐药性。该研究纳入了95例患者，其中5例在我院确诊。接受GC单药治疗的患者与在2周内加用二线治疗的患者的OS无显著差异（100天OS率：86.6%对77.7%；p = 0.338）。此外，在2周内接受GC冲击治疗的患者与未接受该治疗的患者的100天OS也无差异（77.5%对93.1%，p = 0.129）。在多变量分析中，治疗前严重程度评分≥8（风险比[HR]，2.99；95%置信区间[CI] 1.05 - 8.50）和血小板≥6.9×10^4/µL（HR，2.26；95% CI 1.01 - 5.02）与较短的TTNTD显著相关。在超急性期，额外的二线治疗或GC冲击治疗没有优势。较高的严重程度评分和血小板值可能预示对GC治疗的耐药性。
