Chen Can, Chen Kuang, Huang Xilian, Wang Kaile, Qian Shenxian
Department of Hematology, Hangzhou First People's Hospital, Hangzhou, Zhejiang 310006, P.R. China.
Exp Ther Med. 2018 Mar;15(3):2739-2748. doi: 10.3892/etm.2018.5743. Epub 2018 Jan 12.
The current study presents the case of a 9-year-old Chinese boy who presented with eosinophilia and elevated serum levels of immunoglobulin G4 (IgG4). A bone marrow puncture identified an elevated eosinophil rate of 23% (normal range, <5%), which indicated eosinophilia. However, gene analysis, fluorescent hybridization and other examinations, including bone marrow aspiration, blood routine, auto-antibody tests and parasitic and allergens screening, contradicted a diagnosis of secondary or clonal eosinophilia. Furthermore, the patient exhibited multiple lymph node swelling and a lymph biopsy strongly indicted a pathological diagnosis of IgG4-related disease (IgG4-RD). His peripheral blood flow cytometry confirmed an elevated count of plasmablasts, which is specific to IgG4-RD. The patient responded well to therapy with prednisone and remained healthy in all follow-ups. By taking all these factors into consideration, the boy was diagnosed with IgG4-RD. It is difficult to distinguish IgG4-RD from hypereosinophilic syndrome and the potential association between the two remains unclear. However, the present case study serves as a reminder that IgG4-RD may occur in children and medical professionals should not neglect this possibility.
本研究报告了一名9岁中国男孩的病例，该男孩表现为嗜酸性粒细胞增多和血清免疫球蛋白G4（IgG4）水平升高。骨髓穿刺显示嗜酸性粒细胞率升高至23%（正常范围<5%），表明存在嗜酸性粒细胞增多。然而，基因分析、荧光杂交以及其他检查，包括骨髓抽吸、血常规、自身抗体检测以及寄生虫和过敏原筛查，均排除了继发性或克隆性嗜酸性粒细胞增多症的诊断。此外，患者出现多处淋巴结肿大，淋巴结活检强烈提示为IgG4相关疾病（IgG4-RD）的病理诊断。其外周血流式细胞术证实浆母细胞计数升高，这是IgG4-RD的特异性表现。患者对泼尼松治疗反应良好，所有随访中均保持健康。综合考虑所有这些因素，该男孩被诊断为IgG4-RD。IgG4-RD与高嗜酸性粒细胞综合征难以区分，两者之间的潜在关联仍不明确。然而，本病例研究提醒我们，IgG4-RD可能发生在儿童中，医学专业人员不应忽视这种可能性。
