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1例酷似IgG4相关性泪腺炎和涎腺炎的边缘区B细胞淋巴瘤。

A case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis.

作者信息

Ohta Miho, Moriyama Masafumi, Goto Yuichi, Kawano Shintaro, Tanaka Akihiko, Maehara Takashi, Furukawa Sachiko, Hayashida Jun-Nosuke, Kiyoshima Tamotsu, Shimizu Mayumi, Arinobu Yojiro, Nakamura Seiji

机构信息

Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Laboratory of Oral Pathology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Higashi-ku, Fukuoka, Japan.

出版信息

World J Surg Oncol. 2015 Feb 21;13:67. doi: 10.1186/s12957-015-0459-z.

Abstract

BACKGROUND

IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease).

CASE PRESENTATION

We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma.

CONCLUSION

Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

摘要

背景

IgG4相关性泪腺炎和涎腺炎(IgG4-DS),即所谓的米库利奇病,其特征为血清IgG4升高以及腺组织中IgG4阳性浆细胞浸润。最近,多项研究报道了在IgG4相关疾病背景下发生的恶性淋巴瘤以及产生IgG4的恶性淋巴瘤(非IgG4相关疾病)。

病例报告

我们报告了一例70岁男性病例,因其血清IgG4浓度高(215mg/dl)以及腮腺和颌下腺双侧肿大而被高度怀疑为IgG4-DS。对颈部淋巴结和部分颌下腺进行了活检。这些组织病理学结果随后证实诊断为边缘区B细胞淋巴瘤。

结论

IgG4-DS需要与其他疾病进行鉴别诊断,包括干燥综合征、结节病、卡斯特曼病、韦格纳肉芽肿、淋巴瘤和癌症。我们认为对肿胀病变进行活检对于IgG4-DS的明确诊断很重要,并讨论了该病例的发病机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d5d3/4350294/5b5a0e051331/12957_2015_459_Fig1_HTML.jpg

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