Solitary follicular lymphoma with plasmacytic differentiation associated with localized AL amyloidosis in the jejunum
Solitary follicular lymphoma with plasmacytic differentiation associated with localized AL amyloidosis in the jejunum
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孤立性滤泡性淋巴瘤伴浆细胞分化，与空肠局部 AL 淀粉样变性相关
DOI：
10.1007/s00277-023-05207-4
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发表时间：
2023
影响因子：
3.5
通讯作者：
Naiki Hironobu
中科院分区：
文献类型：
--
作者：
Tajima Katsushi;Nomura Takashi;Terada Taichi;Okuyama Shuhei;Kumagai Hiroaki;Maeda Kunihiko;Ogata Shinya;Naiki Hironobu
尊敬的编辑，胃肠道（GI）淀粉样变性根据其基础疾病分为全身性和局部性两种类型[1]。局限型非常罕见，仍是一种有争议的与恶性淋巴瘤相关的临床病理实体，最近提出了意义不明的局限性B细胞瘤形成（BNL）[2-4]。我们报告一个孤立性滤泡性淋巴瘤（FL）伴浆细胞分化和空肠瘤周免疫球蛋白轻链（AL）淀粉样变性的病例。一位患有上腹痛的69岁日本妇女，33年前因卵巢囊肿接受了卵巢切除术，她去了一家诊所。上消化道内视镜检查发现前下胃体的Bormann 4型进展期胃癌。患者转至我院进行手术治疗。术前全结肠镜检查结果显示升结肠和直肠乙状结肠交界处有两个腺瘤。患者接受机器人辅助远端胃切除术治疗。在空肠上偶然发现了一个直径16 mm的孤立性粘膜下肿瘤（SMT），并将其切除以进行Roux-en-Y重建区域（图1a）。术后，胃被诊断为腺癌，淋巴结转移，病理分期IIA。开始辅助化疗，但由于她的不良视力模糊而停止。组织学观察显示，空肠内SMT细胞主要为典型的中心细胞，滤泡间区有大量浆细胞淋巴细胞和少见的Dutcher小体，并有粉红色无定形沉积物（图1a，B，c）。无淋巴上皮病变（补充1）。滤泡细胞对CD 10、CD 20、CD 79 a和Bcl-2呈阳性，对CD 138几乎呈阴性（图1 d、e、f、g、h、i）。浆细胞淋巴细胞、滤泡周围至滤泡间和无定形存款区域的CD 10、CD 79 a、Bcl-2、CD 138和免疫球蛋白λ轻链呈阳性（图1 d、e、g、h、i、j、k），但κ轻链呈阴性（补编1）。免疫球蛋白重链G、A和M的抗体不受限制。无定形粉红色沉积物的刚果红染色呈阳性，交叉偏振光下显示绿-黄-橙子双折射，甲状腺素运载蛋白、淀粉样蛋白AA和β 2-微球蛋白呈阴性（图1 l）（补充资料1c）。采用激光显微切割技术从石蜡包埋的SMT切片中的滤泡区和淀粉样蛋白区提取基因组DNA，并将每个DNA用作扩增免疫球蛋白重链（IGH）基因重排的模板。卵泡区IGH可变连接和多样性连接的表达模式与其在淀粉样蛋白区的表达模式完全相同，表明浆细胞卵泡间细胞通常起源于卵泡中心细胞（补充资料1d）。血清和尿液免疫固定研究对单克隆蛋白呈阴性，游离轻链的κ/λ比值为0.64。正电子发射断层扫描/计算机断层扫描显示她的身体（包括胃肠道）没有增强的FDG摄取。总的来说，患者病理诊断为孤立性FL 1级伴浆细胞分化和局限性λ AL淀粉样变性，根据FLIPI分类风险较低，根据原发性胃肠道淋巴瘤的Lugano分类为I期。患者未接受任何治疗，一年多来一直保持良好状态。
Dear Editor, Gastrointestinal (GI) amyloidosis is classified into systemic and localized types, based on its underlying conditions [1]. The localized type, which is quite rare, remains a debated clinicopathological entity associated with malignant lymphomas and a recently suggested localized B cell neoplasia of undetermined significance (BNL)[2–4]. We describe a case of solitary follicular lymphoma (FL) with plasmacytic differentiation and peritumoral immunoglobulin light chain (AL) amyloidosis in the jejunum. A 69-year-old Japanese woman with epigastralgia, who had undergone an oophorectomy for an ovarian cyst 33 years ago, visited a clinic. Upper endoscopy detected Bormann type 4 advanced gastric cancer in the antero-inferior gastric body. The patient was transferred to our hospital for surgical treatment. Pre-operative total colonoscopy findings showed two adenomas in the ascending colon and rectosigmoid junction. The patient was treated with robotic-assisted distal gastrectomy. A solitary submucosal tumor (SMT), 16 mm in diameter, was incidentally found on the jejunum, which was resected for a Roux-en-Y reconstruction region (Fig. 1 a). Postoperatively, the stomach was diagnosed with adenocarcinoma, lymph node metastasis, and pathological stage IIA. Adjuvant chemotherapy was started, but ceased due to her adverse blurred vision. Histopathological findings of the SMT in the resected jejunum showed that the cells in follicles were mostly classical centrocytes, while there were many plasmacytic lymphocytes and rare Dutcher bodies in the interfollicular areas, adjacent to pink amorphous deposits (Fig. 1 a, b, c). There were no lymphoepithelial lesions (Suppl. 1). The follicle cells were positive for CD10, CD20, CD79a, and Bcl-2 and almost negative for CD138 (Fig. 1 d, e, f, g, h, i). The plasmacytic lymphocytes, and the peri-follicles to interfollicular and amorphous deposit areas, were positive for CD10, CD79a, Bcl-2, CD138, and immunoglobulin lambda light chain (Fig. 1 d, e, g, h, i, j, k), but negative for kappa light chain (Suppl. 1). Antibodies for immunoglobulin heavy chains G, A, and M were not restricted. The amorphous pink deposits were positive for Congo red staining and revealed green-yellow-orange birefringence in cross-polarized light, and negative for transthyretin, amyloid AA, and b2-microglobulin (Fig. 1 l)(Suppl. 1c). Genomic DNA was extracted from the follicle areas and amyloid areas in the paraffin-embedded SMT slide sections by laser microdissection, and each DNA was used as the template for amplification of immunoglobulin heavy chain (IGH) gene rearrangements. The expression patterns of IGH variable-joining and diversityjoining from a follicle area were completely identical to its patterns from an amyloid area, indicating that the plasmacytic interfollicular cells commonly originated from the follicular centrocytes (Suppl. 1d). Serum and urine immunofixation studies were negative for monoclonal protein, and the kappa/lambda ratio of the free light chain was 0.64. Positron emission tomography/computed tomography showed no enhanced FDG uptake in her body, including the GI tract. Collectively, the patient was pathologically diagnosed with solitary FL grade 1 with plasmacytic differentiation and localized lambda AL amyloidosis, with a low risk based on the FLIPI classification, and stage I according to the Lugano classification of primary GI tract lymphoma. The patient received no therapy and remained well for over a year.
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影响因子：
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作者：
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