Modern rheumatology · 2026
Tafro syndrome: early combination therapy outcomes and prolonged thrombocytopenia-a seven-case series.
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Ono Shiro, Nakamura Tomohiro, Nishimura Nobushiro, Matsuoka Hidetoshi, Yoneima Ryo, Kawashima Hiromasa, Miyamoto Makiko, Matsubara Masaki, Yada Noritaka, Yoshimoto Kiyomi
Аннотация
OBJECTIVE: This study aimed to evaluate treatment outcomes, complications, and the clinical course of thrombocytopenia in patients with TAFRO syndrome.
METHODS: We conducted a retrospective analysis of patients treated at Nara Medical University Hospital from 2015 to 2023. Diagnosis of TAFRO syndrome was made according to the 2015 or 2019 criteria, and disease severity was assessed based on the updated disease severity classification. Clinical characteristics and laboratory and histological data were analysed.
RESULTS: Seven patients were included, with four classified as very severe, two as severe, and one as slightly severe. All the patients received early combination therapy (various combinations of glucocorticoids, tocilizumab, cyclosporine, and rituximab), with the second therapy added a median of 5 days after the first. Six patients received eltrombopag. Despite intensive treatment, thrombocytopenia persisted for a prolonged period, with a median time to platelet count normalization of 54 days. Cytomegalovirus reactivation occurred in six patients during treatment but resolved with appropriate antiviral therapy. All the patients achieved remission and survived.
CONCLUSIONS: Thrombocytopenia in TAFRO syndrome persists long term despite intensive combination therapy. Early combination therapy was associated with excellent survival outcomes, suggesting that intensifying treatment solely for thrombocytopenia may not be necessary given its prolonged course.
METHODS: We conducted a retrospective analysis of patients treated at Nara Medical University Hospital from 2015 to 2023. Diagnosis of TAFRO syndrome was made according to the 2015 or 2019 criteria, and disease severity was assessed based on the updated disease severity classification. Clinical characteristics and laboratory and histological data were analysed.
RESULTS: Seven patients were included, with four classified as very severe, two as severe, and one as slightly severe. All the patients received early combination therapy (various combinations of glucocorticoids, tocilizumab, cyclosporine, and rituximab), with the second therapy added a median of 5 days after the first. Six patients received eltrombopag. Despite intensive treatment, thrombocytopenia persisted for a prolonged period, with a median time to platelet count normalization of 54 days. Cytomegalovirus reactivation occurred in six patients during treatment but resolved with appropriate antiviral therapy. All the patients achieved remission and survived.
CONCLUSIONS: Thrombocytopenia in TAFRO syndrome persists long term despite intensive combination therapy. Early combination therapy was associated with excellent survival outcomes, suggesting that intensifying treatment solely for thrombocytopenia may not be necessary given its prolonged course.