Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version
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TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia).
KeywordsThrombocytopenia Anasarca Glucocorticoid Cyclosporin A Tocilizumab
We thank all participants in the All Japan, Ministry of Health, Labor, and Welfare (MHLW) TAFRO Syndrome Team and Castleman Disease Team including for their critical discussion.
Compliance with ethical standards
Sources of support in the form of grants: This work was partially supported by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare (MHLW) of Japan (H27-Nanchi, etc.(Nan)-General-008), and by the Japanese Ministry of Education, Culture, Sports, Science and Technology (Grant No. 17591060 and 15K09510), the Kanazawa Medical University Research Foundation (Grant Nos. S2004-16 and S2007-5), Grant for Assist KAKEN from Kanazawa Medical University (Grant No. K2011-7), Grant for Project Research from High-Tech Research Center of Kanazawa Medical University (Grant No. H2011-11) and Grant for Alumni Research(A) from Kanazawa Medical University (AR2012-06).
Conflict of interest
The authors declare there is no conflict of interest.
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