Response to initial treatment with glucocorticoids in TAFRO syndrome and implications for secondary treatment.

Abstract

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.