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

• DOI: 10.1038/srep42316
• Publication Date: 2017-02-13T10:58:45Z
• Authors: 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
• Keywords: Adult; Male; Castleman Disease; Syndrome; Middle Aged; Article; 3. Good health; Chemokine CXCL10; 03 medical and health sciences; 0302 clinical medicine; Case-Control Studies; Humans; Female; Aged

AbstractMulticentric 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 γ-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.