Ocular adnexal lymphoma associated with IgG4+chronic sclerosing dacryoadenitis: A previously undescribed complication of IgG4-related sclerosing disease
Ocular adnexal lymphoma associated with IgG4+chronic sclerosing dacryoadenitis: A previously undescribed complication of IgG4-related sclerosing disease
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DOI：
10.1097/pas.0b013e31816148ad
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发表时间：
2008-08-01
影响因子：
5.6
通讯作者：
Chan, John K. C.
中科院分区：
文献类型：
--
作者：
Cheuk, Wah;Yuen, Hunter K. L.;Chan, John K. C.
igg4相关的硬化性疾病是最近发现的一种炎性病变，常累及胰腺、颌下腺、泪腺和淋巴结。我们报告3例由igg4相关的慢性硬化性泪腺炎引起的眼附件淋巴瘤，这种现象以前没有报道过。患者表现为双侧或单侧眼附件肿块，通常持续多年。1例患者也有无症状弥漫性淋巴结病。在本次报告之前，有两名患者被活检证实患有IgG4相关的慢性硬化性泪腺炎，而我在之前的鼻咽活检中发现IgG4+细胞增加，这证明了IgG4相关的硬化性疾病的全身累及。2例表现为结外边缘区黏液sa相关淋巴组织型淋巴瘤（1例伴有大细胞转化）和1例滤泡性淋巴瘤。因此，igg4相关硬化疾病的淋巴样增生可为淋巴瘤的发生提供基质。此外，我们报告了3例眼附件结外边缘区b细胞淋巴瘤，其背景表现为硬化性炎症和大量IgG4+单型浆细胞。由于缺乏先前的活组织检查或血清IgG4滴度的信息，尚不清楚这些病例是淋巴瘤合并IgG4相关的硬化疾病还是新生淋巴瘤。然而，这些病例的独特之处在于肿瘤细胞表达IgG4（轻链受限），而未选择的眼附件淋巴瘤病例不表达IgG4。
IgG4-related sclerosing disease is a recently recognized inflammatory lesion frequently involving pancreas, submandibular gland, lacrimal gland, and lymph node. We report 3 cases of ocular adnexal lymphoma arising in IgG4-related chronic sclerosing dacryoadenitis, a phenomenon that has not been previously reported. The patients presented with bilateral or unilateral ocular adnexal mass usually present for many years. One patient also had asymptomatic diffuse lymphadenopathy. Two patients had biopsy-proven IgG4-related chronic sclerosing dacryoadenitis before the current presentation, and I had systemic involvement by IgG4-related sclerosing disease as evidenced by increased IgG4+ cells in a prior nasopharyngeal biopsy. Two cases showed features of extranodal marginal zone lymphoma of muco sa-associated lymphoid-tissue type (I with large cell transformation) and I follicular lymphoma. Thus, the lymphoid hyperplasia of IgG4-related sclerosing disease can provide a substrate for the emergence of lymphoma. In addition, we report 3 cases of ocular adnexal extranodal marginal zone B-cell lymphoma that show sclerosing inflammation in the background and numerous IgG4+ monotypic plasma cells. In the absence of prior biopsies or information on serum IgG4 titer, it is unclear whether these cases represent lymphoma complicating IgG4-related sclerosing disease or de novo lymphoma. Nonetheless, these cases are distinctive in that the neoplastic cells express IgG4 (light chain restricted), whereas unselected cases of ocular adnexal lymphomas do not show IgG4 expression.