PDF(Pubmed)
Abstract：
BACKGROUND: Marginal zone lymphoma (MZL) is an indolent non-Hodgkin B cell lymphoma with various architectural pattern including perifollicular, follicular colonization, nodular, micronodular, and diffuse patterns. A sclerotic variant has not been previously reported and represents a diagnostic pitfall.
METHODS: A 66-year-old male developed left upper extremity swelling. Chest computed tomography (CT) in September 2020 showed 14 cm mass in left axilla. Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic. Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells. Flow cytometry showed a small population of CD5-, CD10-, kappa restricted B cells. Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified. Upon being diagnosed with MZL, patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and achieved complete remission by positron emission tomography/CT.
CONCLUSIONS: This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall. Moreover, this constitutes a new architectural pattern. While sclerotic lymphomas have rarely been described (often misdiagnosed as retroperitoneal fibrosis), we do not know of any cases describing this architectural presentation of MZL.
METHODS: A 66-year-old male developed left upper extremity swelling. Chest computed tomography (CT) in September 2020 showed 14 cm mass in left axilla. Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic. Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells. Flow cytometry showed a small population of CD5-, CD10-, kappa restricted B cells. Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified. Upon being diagnosed with MZL, patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and achieved complete remission by positron emission tomography/CT.
CONCLUSIONS: This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall. Moreover, this constitutes a new architectural pattern. While sclerotic lymphomas have rarely been described (often misdiagnosed as retroperitoneal fibrosis), we do not know of any cases describing this architectural presentation of MZL.
摘要：
背景：边缘区淋巴瘤（MZL）是一种惰性的非霍奇金B细胞淋巴瘤，具有各种结构模式，包括滤泡周，卵泡定植，结节状,微结节,和扩散模式。硬化变体以前没有报道过，代表诊断陷阱。
方法：一名66岁男性出现左上肢肿胀。2020年9月的胸部计算机断层扫描（CT）显示左腋窝有14厘米的肿块。腋窝淋巴结针芯活检显示硬化组织伴不典型B淋巴样浸润，但不能诊断。进行切除活检以进行诊断，并显示广泛的纤维化和浸润B细胞的次要成分。流式细胞术显示少量CD5-，CD10-,κ限制的B细胞。鉴定单克隆免疫球蛋白重链和轻链基因重排。在被诊断为MZL后，患者接受了利妥昔单抗治疗，环磷酰胺,阿霉素,长春新碱,和泼尼松，并通过正电子发射断层扫描/CT获得完全缓解。
结论：这是一个重要的病例报告，因为从形态学上看，该病例很容易被忽视，因为非特异性纤维化伴慢性炎症是一个显著的诊断缺陷。此外，这构成了一种新的建筑模式。虽然很少描述硬化性淋巴瘤（经常误诊为腹膜后纤维化），我们不知道有任何案例描述MZL的这种架构演示。
方法：一名66岁男性出现左上肢肿胀。2020年9月的胸部计算机断层扫描（CT）显示左腋窝有14厘米的肿块。腋窝淋巴结针芯活检显示硬化组织伴不典型B淋巴样浸润，但不能诊断。进行切除活检以进行诊断，并显示广泛的纤维化和浸润B细胞的次要成分。流式细胞术显示少量CD5-，CD10-,κ限制的B细胞。鉴定单克隆免疫球蛋白重链和轻链基因重排。在被诊断为MZL后，患者接受了利妥昔单抗治疗，环磷酰胺,阿霉素,长春新碱,和泼尼松，并通过正电子发射断层扫描/CT获得完全缓解。
结论：这是一个重要的病例报告，因为从形态学上看，该病例很容易被忽视，因为非特异性纤维化伴慢性炎症是一个显著的诊断缺陷。此外，这构成了一种新的建筑模式。虽然很少描述硬化性淋巴瘤（经常误诊为腹膜后纤维化），我们不知道有任何案例描述MZL的这种架构演示。
