Department of Hematology, Jiangxi Provincial Children's Hospital, Nanchang, China.
Department of Rheumatology and Immunology, Jiangxi Provincial Children's Hospital, Nanchang, China.
Front Immunol. 2024 Apr 30;15:1376276. doi: 10.3389/fimmu.2024.1376276. eCollection 2024.
Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition.
A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies.
This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
肝脏炎性假瘤（IPT）是一种罕见的疾病，常被误诊为恶性肿瘤，导致不必要的手术切除。IgG4 相关疾病（IgG4-RD）的新概念已得到广泛认可，包括 IgG4 相关肝 IPT 等疾病。临床上和影像学上，皮质类固醇和免疫抑制疗法已被证明对该疾病有效。
一名 3 岁中国男孩因贫血、原因不明发热和肝区触痛就诊，病史长达 11 个月。血液检查显示慢性贫血（Hb：6.4g/L，MCV：68.6fl，MCH：19.5pg，网织红细胞：1.7%），伴有炎症反应和血清 IgG4 水平升高（1542.2mg/L）。腹部增强 CT 显示右外侧叶 7.6cm 低密度肿块，磁共振成像显示 T1 加权图像稍低信号，T2 加权图像稍高信号，提示肝恶性肿瘤可能。随后的肝活检显示纤维基质和密集的淋巴浆细胞浸润的肿块。免疫组化分析证实 IgG4 阳性浆细胞存在，诊断为 IgG4 相关肝 IPT。皮质类固醇和吗替麦考酚酯治疗后迅速缓解。
本研究强调了肝脏 IPT 的诊断方法，包括组织病理学、免疫组化、影像学、血清学、器官受累和治疗反应。早期组织学检查在临床指导中起着关键作用，可以避免误诊为肝肿瘤和不必要的手术干预。
