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Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version.

Abstract：

: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).

journal_name

Int J Hematol

journal_title

International journal of hematology

authors

Masaki Y,Kawabata H,Takai K,Kojima M,Tsukamoto N,Ishigaki Y,Kurose N,Ide M,Murakami J,Nara K,Yamamoto H,Ozawa Y,Takahashi H,Miura K,Miyauchi T,Yoshida S,Momoi A,Awano N,Ikushima S,Ohta Y,Furuta N,Fujimoto S,Ka

doi

10.1007/s12185-016-1979-1

subject

Has Abstract

pub_date

2016-06-01 00:00:00

pages

686-92

issue

6

eissn

0925-5710

issn

1865-3774

pii

10.1007/s12185-016-1979-1

journal_volume

103

pub_type

杂志文章

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