TAFRO syndrome successfully treated with tocilizumab: A case report and systematic review

Mod Rheumatol. 2018 May;28(3):564-569. doi: 10.3109/14397595.2015.1120389. Epub 2016 Feb 17.

Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is considered as a unique clinicopathologic variant of multicentric Castleman's disease and is recently reported in Japan. This entity represents a severe inflammatory state leading to organ failures such as severe liver dysfunction seen in our case, and can be treated by immunosuppressive agents, steroids, and cyclosporine shown in several case reports. A systematic review and our case suggest the potential utility of tocilizumab as a treatment for TAFRO syndrome.

Keywords: Castleman’s disease; Liver injury; Systematic review; Thrombocytopenia; Tocilizumab; anasarca; and organomegaly syndrome; fever; reticulin fibrosis.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Antibodies, Monoclonal, Humanized / administration & dosage
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Castleman Disease / drug therapy*
  • Edema / drug therapy*
  • Female
  • Fever / drug therapy*
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use*
  • Middle Aged
  • Syndrome
  • Thrombocytopenia / drug therapy*

Substances

  • Antibodies, Monoclonal, Humanized
  • Immunosuppressive Agents
  • tocilizumab