Research Papers: Pathology:

Idiopathic multicentric Castleman’s disease: a clinicopathologic study in comparison with IgG4-related disease

Kyoko Otani, Dai Inoue, Kohei Fujikura, Takahiro Komori, Shiho Abe-Suzuki, Takuma Tajiri, Tomoo Itoh and Yoh Zen _

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Oncotarget. 2018; 9:6691-6706. https://doi.org/10.18632/oncotarget.24068

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Kyoko Otani1, Dai Inoue2, Kohei Fujikura1, Takahiro Komori2, Shiho Abe-Suzuki1, Takuma Tajiri3, Tomoo Itoh1 and Yoh Zen1

1Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan

2Department of Radiology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

3Department of Diagnostic Pathology, Tokai University Hachioji Hospital, Tokyo, Japan

Correspondence to:

Yoh Zen, email: [email protected]

Keywords: Castleman; IL-6; IgG4; autoimmune pancreatitis; pathology

Received: October 30, 2017    Accepted: January 03, 2018    Published: January 09, 2018


The present study aimed to compare clinicopathologic features between idiopathic multicentric Castleman’s disease (n=22) and IgG4-related disease (n=26). Histology was analyzed using lymph node and lung biopsies. The expression of IL-6 mRNA in tissue was also examined by in situ hybridization and real-time PCR. Patients with idiopathic multicentric Castleman’s disease were significantly younger than those with IgG4-related disease (p<0.001). Splenomegaly was observed in only idiopathic multicentric Castleman’s disease (p=0.002), while pancreatitis and sialo-dacryoadenitis were restricted to IgG4-related disease (both p<0.001). Serum IgG4 concentrations were commonly elevated at >135 mg/dL in both groups (p=0.270). However, the IgG4/IgG ratio in IgG4-related disease was significantly higher than that in Castleman’s disease (p<0.001). Histologically, sheet-like plasmacytosis was highly characteristic of idiopathic multicentric Castleman’s disease (p<0.001), while plasmacytic infiltration in IgG4-related disease was always associated with intervening lymphocytes. Similar to laboratory findings, the IgG4/IgG-positive plasma cell ratio, but not the IgG4-positive cell count, was significantly higher in IgG4-related disease (p=0.002). Amyloid-like hyalinized fibrosis was found in 6/8 lung biopsies (75%) of Castleman’s disease. The over-expression of IL-6 mRNA was not confirmed in tissue samples of Castleman’s disease by either in situ hybridization or quantitative real-time PCR. In conclusion, useful data for a differential diagnosis appear to be age, affected organs, the serum IgG4/IgG ratio, sheet-like plasmacytosis in biopsies, and the IgG4/IgG-positive cell ratio on immunostaining. Since IL-6 was not over-expressed in tissue of idiopathic multicentric Castleman’s disease, IL-6 may be produced outside the affected organs, and circulating IL-6 may lead to lymphoplasmacytosis at nodal and extranodal sites.

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