TAFRO Syndrome

Takuro Igawa; Yasuharu Sato

doi:10.1016/j.hoc.2017.09.009

TAFRO Syndrome

Takuro Igawa, Yasuharu Sato

Research output: Contribution to journal › Review article › peer-review

34 Citations (Scopus)

Abstract

TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment.

Original language	English
Pages (from-to)	107-118
Number of pages	12
Journal	Hematology/Oncology Clinics of North America
Volume	32
Issue number	1
DOIs	https://doi.org/10.1016/j.hoc.2017.09.009
Publication status	Published - Feb 2018

Keywords

Castleman disease
Clinical features
Pathogenesis
TAFRO syndrome

ASJC Scopus subject areas

Hematology
Oncology

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10.1016/j.hoc.2017.09.009

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title = "TAFRO Syndrome",

abstract = "TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment.",

keywords = "Castleman disease, Clinical features, Pathogenesis, TAFRO syndrome",

author = "Takuro Igawa and Yasuharu Sato",

note = "Funding Information: This work was partially supported by a Grant-in-Aid for Scientific Research (C) ( JSPS KAKENHI Grant Number JP16K08666 ) from the Japan Society for the Promotion of Science. Publisher Copyright: {\textcopyright} 2017 Elsevier Inc. Copyright: Copyright 2017 Elsevier B.V., All rights reserved.",

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doi = "10.1016/j.hoc.2017.09.009",

language = "English",

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AU - Igawa, Takuro

AU - Sato, Yasuharu

N1 - Funding Information: This work was partially supported by a Grant-in-Aid for Scientific Research (C) ( JSPS KAKENHI Grant Number JP16K08666 ) from the Japan Society for the Promotion of Science. Publisher Copyright: © 2017 Elsevier Inc. Copyright: Copyright 2017 Elsevier B.V., All rights reserved.

PY - 2018/2

Y1 - 2018/2

N2 - TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment.

AB - TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment.

KW - Castleman disease

KW - Clinical features

KW - Pathogenesis

KW - TAFRO syndrome

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TAFRO Syndrome

Abstract

Keywords

ASJC Scopus subject areas

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