Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

Intern Med. 2017 Aug 15;56(16):2205-2211. doi: 10.2169/internalmedicine.8522-16. Epub 2017 Aug 1.

Abstract

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, pleural effusion, fever, renal dysfunction, reticulin fibrosis of the bone marrow, and organomegaly. The clinical course varies significantly among patients. However, the prognosis is usually dismal in patients with severe TAFRO syndrome, and no optimal treatment has yet been established. We herein describe the first case of TAFRO syndrome, which was successfully treated with combination therapy consisting of tocilizumab, prednisone, and cyclophosphamide.

Keywords: TAFRO syndrome; cyclophosphamide; tocilizumab.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Bone Marrow / pathology
  • Cyclophosphamide / therapeutic use*
  • Drug Therapy, Combination
  • Edema / drug therapy
  • Fever / drug therapy
  • Fibrosis
  • Glucocorticoids / therapeutic use
  • Humans
  • Lymphadenopathy / diagnostic imaging
  • Lymphadenopathy / drug therapy
  • Male
  • Pleural Effusion / diagnostic imaging
  • Pleural Effusion / drug therapy
  • Prednisone / therapeutic use*
  • Syndrome
  • Thrombocytopenia / drug therapy*
  • Tomography, X-Ray Computed

Substances

  • Antibodies, Monoclonal, Humanized
  • Glucocorticoids
  • Cyclophosphamide
  • tocilizumab
  • Prednisone