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一例与滤泡性淋巴瘤伴浆细胞分化相关的 AL 淀粉样变性。

A case of AL amyloidosis associated with follicular lymphoma with plasmacytic differentiation.

机构信息

Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital, 15-749, Honmachi, Higashiyama-ku, Kyoto, 605-8981, Japan.

Department of Hematology, Aiseikai Yamashina Hospital, Kyoto, Japan.

出版信息

Int J Hematol. 2020 Feb;111(2):317-323. doi: 10.1007/s12185-019-02734-7. Epub 2019 Sep 21.

DOI:10.1007/s12185-019-02734-7
PMID:31542852
Abstract

A 58-year-old woman underwent emergency surgical resection of the small intestine for intussusception as diagnosed at our hospital. Histopathological diagnosis of the resected specimen of the ileum was amyloid light chain (AL) amyloidosis. The colonoscopy after the surgical resection and following histopathological analysis of the biopsied specimens of the colon revealed follicular lymphoma (FL) grade 1 with plasmacytic differentiation. Histological findings of these ileal and colonic lesions were characteristic. In the ileum, CD10-positive lymphoid follicles and CD38-positive interfollicular plasma cell infiltration into villi were detected. The amyloid deposition was localized to the ileum and was adjacent to lymphoid follicles and interfollicular plasma cells. Furthermore, fluorescence in situ hybridization (FISH) for paraffin-embedded tissue sections (tissue-FISH) revealed that both the B cells in follicular lesions and the interfollicular plasma cells showed IGH/BCL2 fusion signals, which means the interfollicular plasma cells were originated from the differentiated neoplastic follicular B cells. The patient was treated with six courses of lymphoma chemotherapy and attained complete remission without any symptoms associated with amyloidosis. Further case analyses are needed to clarify the clinicopathological findings and to establish therapeutic strategy of AL amyloidosis associated with FL and FL with plasmacytic differentiation.

摘要

一位 58 岁女性因我院诊断的肠套叠而行小肠急诊切除术。回肠切除标本的组织病理学诊断为淀粉样轻链(AL)淀粉样变性。手术后结肠镜检查和结肠活检标本的组织病理学分析显示 1 级滤泡性淋巴瘤(FL)伴浆细胞分化。这些回肠和结肠病变的组织学表现具有特征性。在回肠中,检测到 CD10 阳性的淋巴滤泡和 CD38 阳性的滤泡间浆细胞浸润绒毛。淀粉样物质沉积局限于回肠,与淋巴滤泡和滤泡间浆细胞相邻。此外,石蜡包埋组织切片的荧光原位杂交(tissue-FISH)显示滤泡病变中的 B 细胞和滤泡间浆细胞均显示IGH/BCL2 融合信号,这意味着滤泡间浆细胞起源于分化的肿瘤性滤泡 B 细胞。该患者接受了六轮淋巴瘤化疗,完全缓解,无任何与淀粉样变性相关的症状。需要进一步的病例分析来阐明与 FL 相关的 AL 淀粉样变性和伴有浆细胞分化的 FL 的临床病理特征,并制定治疗策略。

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本文引用的文献

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原发性大脑免疫球蛋白轻链淀粉样变瘤一例合并多发性硬化症。
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Ann Hematol. 2023 Jun;102(6):1625-1627. doi: 10.1007/s00277-023-05207-4. Epub 2023 Apr 5.
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