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The following is a summary of “Response to initial treatment with glucocorticoids in TAFRO syndrome and implications for secondary treatment,” published in the January 2025 issue of Hematology by Tominaga et al.
TAFRO syndrome is a rare condition characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. The therapeutic effect of initial treatments with glucocorticoid (GC) therapy remains unclear.
Researchers conducted a retrospective study to investigate the therapeutic effect of various initial treatments incorporating GC in TAFRO syndrome.
They retrospectively collected cases of TAFRO syndrome up to November 2023. Overall survival (OS) and resistance to GC therapy were assessed, with resistance analyzed based on time to the next treatment or death (TTNTD). The study included 95 patients, including 5 diagnosed at our hospital.
The results showed OS did not differ significantly between patients who received GC monotherapy and those with second line (2L) therapy added within 2 weeks (100-day OS rate: 86.6% vs 77.7%; P = 0.338). There was no significant difference in 100-day OS between patients who received GC pulse therapy within 2 weeks and those who did not (77.5% vs 93.1%, P = 0.129). Multivariate analyses showed pretreatment severity score ≥8 (HR, 2.99; 95% CI 1.05–8.50) and platelets ≥6.9 × 104/µL (HR, 2.26; 95% CI 1.01–5.02) were significantly associated with shorter TTNTD.
Investigators found no advantage of additional second-line or GC pulse therapy in the hyperacute phase. Higher severity scores and platelet values predicted GC therapy resistance.
Source: link.springer.com/article/10.1007/s12185-025-03933-1
