TAFRO syndrome: A disease that known is half cured

Hematol Oncol. 2023 Aug;41(3):310-322. doi: 10.1002/hon.3075. Epub 2022 Oct 3.

Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is rare in clinical practice. It is a systemic inflammatory disease caused by a cytokine storm. Its clinical manifestations include thrombocytopenia, systemic edema, fever, bone marrow fibrosis, renal insufficiency, and organ enlargement. The high mortality rate of TAFRO syndrome is due to the difficulty of acquiring biopsy samples for diagnosis and the rapid disease progression. This disease is poorly understood by clinicians. Early detection, accurate diagnosis, and timely treatment play key roles in prolonging the survival of the patients. This review summarizes the latest progress in the pathogenesis, diagnostic criteria, and treatment regimens of TAFRO syndrome, aiming to help clinicians better understand TAFRO syndrome and improve its diagnosis and treatment.

Keywords: TAFRO syndrome; clinical diagnostic criteria; cytokine storm; etiology; pathogenesis; treatment management.

Publication types

  • Review

MeSH terms

  • Anemia*
  • Castleman Disease* / drug therapy
  • Castleman Disease* / therapy
  • Edema / diagnosis
  • Edema / drug therapy
  • Edema / etiology
  • Humans
  • Primary Myelofibrosis* / drug therapy
  • Renal Insufficiency* / diagnosis
  • Renal Insufficiency* / drug therapy
  • Thrombocytopenia* / diagnosis
  • Thrombocytopenia* / etiology
  • Thrombocytopenia* / therapy

Supplementary concepts

  • Multi-centric Castleman's Disease