A Case of Rosai-Dorfman Disease Mimicking Immunoglobulin G4?Related Autoimmune Pancreatitis
A Case of Rosai-Dorfman Disease Mimicking Immunoglobulin G4?Related Autoimmune Pancreatitis
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类似免疫球蛋白G4相关的Rosai-Dorfman病一例自身免疫性胰腺炎
DOI：
10.1097/mpa.0000000000001945
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发表时间：
2021
期刊：
影响因子：
2.9
通讯作者：
Joshi Deepak
中科院分区：
文献类型：
--
作者：
Jheeta Anup Singh;Nayagam Jeremy S.;Zen Yoh;Joshi Deepak
致编辑：Rosai-Dorfman病（RDD），也称为窦组织细胞增生症伴巨大淋巴结病，是一种病因不明的良性疾病，通常表现为无痛性巨大淋巴结病，并可有淋巴结表现。1 Rosai-Dorfman病是免疫球蛋白G4相关疾病（IgG 4-RD）背景下的一种重要疾病模拟物。它在儿童和年轻成年男性中最常见，并优先影响非洲裔人。我们报告一例RDD模仿IgG 4相关的1型自身免疫性胰腺炎（1型AIP），这是保守治疗。本文对RDD的诊断特点及治疗进行了讨论。一位78岁的白色男性，有3周的间歇性上腹痛病史，并伴有不明原因的体重减轻。无黄疸、大便苍白或尿色深的病史。肝脏生化正常（胆红素5 mg/dL，天冬氨酸转氨酶17 IU/L，碱性磷酸酶104 IU/L，γ-谷氨酰转移酶22 U/L），血清碳水化合物抗原19-9为21 U/mL。计算机断层扫描（CT）和内镜超声显示胰头肿瘤伴远端胰管扩张。两个单独的CT引导活检报告了纤维炎症过程，没有恶性肿瘤的证据。胰腺组织被淋巴细胞和浆细胞大量浸润（图1A和1B）。1A、B）。嗜酸性粒细胞和中性粒细胞不明显。不规则的纤维化是另一个特征，但不是特别的故事状模式。未发现闭塞性静脉炎。与典型的1型AIP不同，鉴定了巨噬细胞聚集灶，但其意义尚不清楚（图1C）。免疫染色显示许多IgG 4+浆细胞浸润，每个高倍视野多达46个细胞（HPF;图1D）。由于强背景IgG免疫染色，无法计算IgG 4 +/IgG+浆细胞比。组织检查后测量的血清IgG 4浓度升高（236 mg/dL;参考范围，0-130 mg/dL）。在多学科会议讨论后，基于1型AIP伴炎性假瘤的推定诊断，开始了泼尼松龙疗程。预计1型AIP对皮质类固醇有显著反应，并形成广泛接受的诊断标准的一部分。2然而，尽管与类固醇治疗一致，重复CT成像并未显示胰腺肿块大小的变化。因此，考虑了其他诊断。对活检标本的重新审查揭示了一些巨噬细胞中的emperipolesis（组织细胞胞质内的炎性细胞），这提高了RDD的可能性。此外，免疫染色
To the Editor: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign condition of unknown etiology, which commonly presents with painless massive lymphadenopathy and can have extranodal manifestations. 1 Rosai-Dorfman disease is emerging as an important disease mimic in the context of immunoglobulin G4–related disease (IgG4-RD). It is most common in children and young adult males and preferentially affects those of African descent. We report a case of RDD mimicking IgG4-related type 1 autoimmune pancreatitis (type 1 AIP), which was treated conservatively. The diagnostic features and treatment of RDD are discussed. A 78-year-old White man presented with a 3-week history of intermittent epigastric pain radiating to his back, associated with unexplained weight loss. There was no history of jaundice, pale stools, or dark urine. Liver biochemistry was normal (bilirubin 5 mg/dL, aspartate aminotransferase 17 IU/L, alkaline phosphatase 104 IU/L, γ-glutamyl transferase 22 U/L), and serum carbohydrate antigen 19-9 was 21 U/mL. Computed tomography (CT) and endoscopic ultrasound demonstrated a tumor in the pancreatic head with distal pancreatic duct dilatation. Two separate CT-guided biopsies reported a fibroinflammatory process with no evidence of malignancy. Pancreatic tissue was massively infiltrated by lymphocytes and plasma cells (Figs. 1A, B). Eosinophils and neutrophils were not conspicuous. Irregular fibrosis was another feature, but not particularly in a storiform pattern. Obliterative phlebitis was not identified. Unlike typical type 1 AIP, foci of macrophage aggregation were identified, but its significance was unclear (Fig. 1C). Immunostaining demonstrated infiltration of many IgG4+ plasma cells, up to 46 cells per high-power field (HPF; Fig. 1D). The IgG4+/IgG+ plasma cell ratio could not be calculated because of strong background IgG immunostaining. Serum IgG4 concentrations measured after the tissue examination were elevated (236 mg/dL; reference range, 0–130 mg/dL). Following discussion in the multidisciplinary meeting, a course of prednisolone was commenced based on a presumptive diagnosis of type 1 AIP with an inflammatory pseudotumor. A dramatic response to corticosteroids is expected in type 1 AIP and forms part of a widely accepted diagnostic criteria. 2 However, despite concordance with steroid therapy, repeat CT imaging did not demonstrate change in the size of the pancreatic mass. Alternative diagnoses were therefore considered. Re-review of the biopsy specimens unveiled emperipolesis (inflammatory cells within the histiocyte cytoplasm) in some macrophages, which raised the possibility of RDD. In addition, immunostaining