Elevated serum interferon Î 3-induced protein 10 kDa is associated with TAFRO syndrome

  • Noriko Iwaki
  • , Yuka Gion
  • , Eisei Kondo
  • , Mitsuhiro Kawano
  • , Taro Masunari
  • , Hiroshi Moro
  • , Koji Nikkuni
  • , Kazue Takai
  • , Masao Hagihara
  • , Yuko Hashimoto
  • , Kenji Yokota
  • , Masataka Okamoto
  • , Shinji Nakao
  • , Tadashi Yoshino
  • , Yasuharu Sato

Research output: Contribution to journalArticlepeer-review

63 Citations (Scopus)

Abstract

Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established. We retrospectively compared levels of serum interferon Î 3-induced protein 10 kDa (IP-10), platelet-derived growth factor (PDGF)-AA, interleukin (IL)-10, and other cytokines between 11 cases of TAFRO-iMCD, 6 cases of plasma cell type iMCD, and 21 healthy controls. During flare-ups, patients with TAFRO-iMCD had significantly higher serum IP-10 and tended to have lower PDGF-AA levels than the other 2 groups. In addition, serum IL-10, IL-23, and vascular endothelial growth factor-A were elevated in both TAFRO-iMCD and iMCD. Elevated serum IP-10 is associated with inflammatory diseases including infectious diseases. There was a strong correlation between high serum IP-10 and the presence of TAFRO-iMCD, suggesting that IP-10 might be involved in the pathogenesis of TAFRO-iMCD.

Original languageEnglish
Article number42316
JournalScientific reports
Volume7
DOIs
Publication statusPublished - 13-02-2017
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • General

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