8 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 languageEnglish
Pages (from-to)107-118
Number of pages12
JournalHematology/Oncology Clinics of North America
Volume32
Issue number1
DOIs
Publication statusPublished - Feb 1 2018

Keywords

  • Castleman disease
  • Clinical features
  • Pathogenesis
  • TAFRO syndrome

ASJC Scopus subject areas

  • Hematology
  • Oncology

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