Hematopathology Service.
Department of Pathology, Amsterdam UMC, location VU University Medical Center, Amsterdam, The Netherlands.
Am J Surg Pathol. 2021 Jul 1;45(7):939-944. doi: 10.1097/PAS.0000000000001684.
Immunoglobulin light chain (AL) amyloidosis is characterized by the deposition of amyloid fibers derived from pathologic immunoglobulin light chains. Although systemic plasma cell neoplasms are the most common cause of AL amyloidosis, a subset of cases is caused by B-cell lymphoproliferative disorders such as lymphoplasmacytic lymphoma or extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. Recently, SOX11-negative IGH hypermutated mantle cell lymphoma (MCL) is recognized to show frequent plasmacytic differentiation and indolent clinical course. Here, we report 3 cases of peritumoral AL amyloidosis associated with SOX11-negative MCL. All 3 cases showed cyclin D1 expression by immunohistochemistry and CCND1 translocation as detected by fluorescence in situ hybridization analysis. Peritumoral AL amyloidosis was observed at the biopsy sites in the gastrointestinal tract, a supraclavicular lymph node, and a cervical lymph node, and all presented with marked plasmacytic differentiation of lymphoma cells. None of the cases showed evidence of bone marrow involvement by morphology and immunophenotyping. None of the patients had distant organ involvement with systemic amyloidosis. All 3 patients had an indolent clinical course and are alive with disease at the time of the last follow-up (range: 48 to 74 mo). Our findings show that MCL with plasmacytic differentiation can cause amyloid deposition and CCND1 abnormalities should be performed in all cases of extramedullary AL amyloidosis. Recognition of indolent MCL as a cause of peritumoral AL amyloidosis may have important clinical management implications.
免疫球蛋白轻链（AL）淀粉样变性的特征是由病理性免疫球蛋白轻链衍生的淀粉样纤维沉积。尽管系统性浆细胞肿瘤是 AL 淀粉样变性的最常见原因，但一部分病例是由 B 细胞淋巴增生性疾病引起的，如淋巴浆细胞淋巴瘤或黏膜相关淋巴组织结外边缘区淋巴瘤。最近，SOX11 阴性的IGH 高突变性套细胞淋巴瘤（MCL）被认为具有频繁的浆细胞分化和惰性的临床病程。在此，我们报告 3 例与 SOX11 阴性 MCL 相关的瘤周 AL 淀粉样变性。所有 3 例均通过免疫组化显示 cyclin D1 表达，通过荧光原位杂交分析检测到 CCND1 易位。瘤周 AL 淀粉样变性发生在胃肠道、锁骨上淋巴结和颈部淋巴结的活检部位，所有部位均表现为淋巴瘤细胞明显的浆细胞分化。形态学和免疫表型均未显示骨髓受累的证据。无患者有远处器官受累伴全身性淀粉样变性。所有 3 例患者均具有惰性的临床病程，在最后一次随访时仍处于疾病状态（范围：48 至 74 个月）。我们的研究结果表明，具有浆细胞分化的 MCL 可引起淀粉样物质沉积，应在所有结外 AL 淀粉样变性病例中进行 CCND1 异常检测。认识到惰性 MCL 是瘤周 AL 淀粉样变性的原因可能对临床管理具有重要意义。
