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 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.
Highlights
Multicentric Castleman disease (MCD) is a rare inflammatory disorder often characterized by inflammatory flare-ups including episodes of systemic inflammation
In a previous prospective study, we reported the clinicopathological analysis of 25 cases of a unique subtype of idiopathic MCD (iMCD) characterized symptomatically by thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O), and known as TAFRO syndrome (TAFRO-iMCD)[10]
Patients with TAFRO-iMCD exhibit thrombocytopenia without polyclonal hypergammaglobulinemia[10,11,12]. These findings suggest that elevated serum IL-6 might not be a primary pathological driver of the proinflammatory hypercytokinemia observed in patients with TAFRO-iMCD
Summary
Multicentric Castleman disease (MCD) is a rare inflammatory disorder often characterized by inflammatory flare-ups including episodes of systemic inflammation. It may be characterized by fever, cachexia, systemic lymphadenopathy, polyclonal hypergammaglobulinemia, microcytic anemia, hypoalbuminemia, and elevated serum inflammatory proteins, such as C-reactive protein (CRP)[1,2,3]. Patients with TAFRO-iMCD exhibit thrombocytopenia without polyclonal hypergammaglobulinemia[10,11,12] These findings suggest that elevated serum IL-6 might not be a primary pathological driver of the proinflammatory hypercytokinemia observed in patients with TAFRO-iMCD. In the present study, to identify the cytokine profile associated with TAFRO-iMCD, we compared serum cytokine profiles between patients with non-HHV-8-associated MCD, including TAFRO-iMCD and plasma cell iMCD (iMCD-NOS), and healthy subjects
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