Lymph Nodes With Increased IgG4-positive Plasma Cells and Patterns Suspicious for IgG4-related Disease: Can Lymph Nodes Be the Only Site of Disease?

Ying Ren Chen, Shan Chi Yu, Ren Ching Wang, Chih Ling Lee, Hsiang Lin Song, L. Jeffrey Medeiros, Chung Tai Yue, Kung Chao Chang

研究成果: Article同行評審

1 引文 斯高帕斯(Scopus)


Lymphadenopathy with increased immunoglobulin (Ig) G4+ plasma cells can be a nonspecific finding or a manifestation of immunoglobulin G4-related disease (IgG4-RD). It remains unclear whether there are characteristic pathologic features of IgG4-RD involving lymph nodes, or if IgG4-RD lymphadenopathy can occur without other manifestations of IgG4-RD. In this study, we assessed 55 lymph node biopsy specimens (44 men and 11 women with a mean age of 55 y) with increased IgG4+ plasma cells that had 1 of the 6 well-described pathologic patterns. We also correlated these findings with IgG4 serum levels and followed these patients for 7 to 108 months (mean, 34.9 mo) for the occurrence of extranodal IgG4-RD. We further compared lymphadenopathy in patients who developed other manifestations of IgG4-RD (RD+, n=20, 36%) versus those who did not (RD-, n=35, 64%). We found that there were only minor significant differences between 2 groups, including frequency of receiving treatment (RD+, 90% vs. RD-, 60%, P=0.021) and higher serum levels of C-reactive protein (>8 mg/L, RD+, 53% vs. RD-, 13%, P=0.007). Other differences were either borderline or not significant, including mean age (RD+, 59.8 y vs. RD-, 51.9 y, P=0.097), male-to-female ratio (RD+, 16:4 vs. RD-, 28:7, P=1), constitutional symptoms (RD+, 25% vs. RD-, 9%, P=0.096), multiple enlarged lymph nodes (RD+, 45% vs. RD-, 26%, P=0.143), good response to therapy (RD+, 94% vs. RD-, 94%, P=1); higher serum IgG4 levels (>280 mg/dL, RD+, 75% vs. RD-, 51%, P=0.086), anemia (RD+, 45% vs. RD-, 43%, P=0.877), leukopenia (RD+, 0% vs. RD-, 3%, P=0.446), thrombocytopenia (RD+, 10% vs. RD-, 6%, P=0.556), positivity for antinuclear antibody (RD+, 24% vs. RD-, 29%, P=0.688), elevated serum levels of lactate dehydrogenase (>225 U/L, RD+, 0% vs. RD-, 20%, P=0.064), elevated serum IgE level (>100 IU/mL, RD+, 75% vs. RD-, 92%, P=0.238), and hypergammaglobulinemia (RD+, 90% vs. RD-, 86%, P=0.754). There were also no differences in morphologic patterns (P=0.466), IgG4+ cell location (P=0.104), eosinophil counts (RD+, 10.3±11.3 vs. RD-, 13.4±17.5, P=0.496), Epstein-Barr virus positivity (RD+, 35% vs. RD-, 60%, P=0.074), and Epstein-Barr virus-positive cell location (P=0.351). Our findings suggest that there are minimal differences between stringently defined IgG4-RD lymphadenopathy with versus without other manifestations of IgG4-RD. These findings also suggest the existence of IgG4-RD lymphadenopathy as the sole presentation of IgG4-RD.

頁(從 - 到)387-396
期刊American Journal of Surgical Pathology
出版狀態Published - 2023 3月 1

All Science Journal Classification (ASJC) codes

  • 解剖學
  • 手術
  • 病理學與法醫學


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