: TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly) syndrome is a rare, life-threatening inflammatory condition linked to infections, neoplasms, and idiopathic multicentric Castleman disease. Interlukin (IL)-6 inhibitors are the primary treatment, but refractory cases require alternatives. This study reports the first two pediatric TAFRO cases successfully treated with anakinra, an IL-1 receptor antagonist. Both patients had severe, rapidly progressing disease with multiorgan involvement. Anakinra, combined with corticosteroids, led to significant improvement and remission. We provide a literature review of pediatric TAFRO, confirming its rarity and the partial efficacy of IL-6 inhibitors in many cases.
Efficacy of High-Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review
Palmeri, Serena;Ferro, Jacopo;Natoli, Valentina;Matucci-Cerinic, Caterina;Papa, Riccardo;Rosina, Silvia;Sorrentino, Stefania;Volpi, Stefano;Vellone, Valerio Gaetano;Gattorno, Marco;Caorsi, Roberta
2025-01-01
Abstract
: TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly) syndrome is a rare, life-threatening inflammatory condition linked to infections, neoplasms, and idiopathic multicentric Castleman disease. Interlukin (IL)-6 inhibitors are the primary treatment, but refractory cases require alternatives. This study reports the first two pediatric TAFRO cases successfully treated with anakinra, an IL-1 receptor antagonist. Both patients had severe, rapidly progressing disease with multiorgan involvement. Anakinra, combined with corticosteroids, led to significant improvement and remission. We provide a literature review of pediatric TAFRO, confirming its rarity and the partial efficacy of IL-6 inhibitors in many cases.
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