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首页> 外文期刊>Clinical Medicine Insights: Blood Disorders >Transformation of Follicular Lymphoma to a High-Grade B-Cell Lymphoma With MYC and BCL2 Translocations and Overlapping Features of Burkitt Lymphoma and Acute Lymphoblastic Leukemia: A Case Report and Literature Review
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Transformation of Follicular Lymphoma to a High-Grade B-Cell Lymphoma With MYC and BCL2 Translocations and Overlapping Features of Burkitt Lymphoma and Acute Lymphoblastic Leukemia: A Case Report and Literature Review

机译：滤泡性淋巴瘤向MYC和BCL2易位并具有伯基特淋巴瘤和急性淋巴细胞白血病的重叠特征的高级B细胞淋巴瘤的转化：病例报告和文献综述

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摘要

Most commonly, histologic transformation (HT) from follicular lymphoma (FL) manifests as a diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS). Less frequently, HT may result in a high-grade B-cell lymphoma (HGBL) with MYC and B-cell lymphoma protein 2 (BCL2) and/or BCL6 gene rearrangements, also known as “double-hit” or “triple-hit” lymphomas. In the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms, the category B-cell lymphoma, unclassifiable was eliminated due to its vague criteria and limiting diagnostic benefit. Instead, the WHO introduced the HGBL category, characterized by MYC and BCL2 and/or BCL6 rearrangements. Cases that present as an intermediate phenotype of DLBCL and Burkitt lymphoma (BL) will fall within this HGBL category. Very rarely, HT results in both the intermediate DLBCL and BL phenotypes and exhibits lymphoblastic features, in which case the WHO recommends that this morphologic appearance should be noted. In comparison with de novo patients with DLBCL, NOS, those with MYC and BCL2 and/or BCL6 gene rearrangements have a worse prognosis. A 63-year-old woman presented with left neck adenopathy. Laboratory assessments, including complete blood count, complete metabolic panel, serum lactate dehydrogenase, and β2-microglobulin, were all normal. A whole-body computerized tomographic (CT) scan revealed diffuse adenopathy above and below the diaphragm. An excisional node biopsy showed grade 3A nodular FL. The Ki67 labeling index was 40% to 50%. A bone marrow biopsy showed a small focus of paratrabecular CD20+ lymphoid aggregates. She received 6 cycles of bendamustine (90 mg/m2 on days +1 and +2) and rituximab (375 mg/m2 on day +2), with each cycle delivered every 4 weeks. A follow-up CT scan at completion of therapy showed a partial response with resolution of axillary adenopathy and a dramatic shrinkage of the large retroperitoneal nodes. After 18 months, she had crampy abdominal pain in the absence of B symptoms. Positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-d-glucose integrated with CT (18F-FDG PET/CT) scan showed widespread adenopathy, diffuse splenic involvement, and substantial marrow involvement. Biopsy of a 2.4-cm right axillary node (SUVmax of 16.1) showed involvement by grade 3A FL with a predominant nodular pattern of growth. A bone marrow biopsy once again showed only a small focus of FL. She received idelalisib (150 mg twice daily) and rituximab (375 mg/m2, monthly) beginning May 2015. After 4 cycles, a repeat CT scan showed a complete radiographic response. Idelalisib was subsequently held while she received corticosteroids for immune-mediated colitis. A month later, she restarted idelalisib with a 50% dose reduction. After 2 weeks, she returned to clinic complaining of bilateral hip and low lumbar discomfort but no B symptoms. A restaging 18F-FDG PET/CT in January 2016 showed dramatic marrow uptake. A bone marrow aspirate showed sheets of tumor cells representing a spectrum from intermediate-sized cells with lymphoblastic features to very large atypical cells with multiple nucleoli. Two distinct histologies were present; one remained consistent with the patient’s known FL with a predominant nodular pattern and the other consistent with HT (the large atypical cells expressed PAX5, CD10, BCL2, and c-MYC and were negative for CD20, MPO, CD34, CD30, and BCL6). Focal areas showed faint, heterogeneous expression of terminal deoxynucleotidyl transferase best seen on the clot section. Ki67 proliferation index was high (4+/4). Fluorescence in situ hybridization analysis showed 2 populations with MYC amplification and/or rearrangement and no evidence of BCL6 rearrangement; a karyotype analysis showed a complex abnormal female karyotype with t(14;18) and multiple structural and numerical abnormalities. She started dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin with concomitant prophylacti
机译：最常见的是，滤泡性淋巴瘤（FL）的组织学转化（HT）表现为弥漫性大B细胞淋巴瘤，除非另有说明（DLBCL，NOS）。 HT较少会导致伴有MYC和B细胞淋巴瘤蛋白2（BCL2）和/或BCL6基因重排的高级别B细胞淋巴瘤（HGBL），也称为“双击”或“三击”淋巴瘤。在2016年世界卫生组织（WHO）淋巴瘤分类的修订版中，由于其模糊的标准和有限的诊断价值，消除了无法分类的B细胞淋巴瘤。相反，WHO引入了HGBL类别，其特征是MYC和BCL2和/或BCL6重排。表现为DLBCL和伯基特淋巴瘤（BL）的中间表型的病例将属于此HGBL类别。极少数情况下，HT会同时导致DLBCL和BL中间表型，并表现出淋巴母细胞特征，在这种情况下，WHO建议应注意这种形态。与从头患有DLBCL，NOS的患者相比，患有MYC和BCL2和/或BCL6基因重排的患者的预后较差。一名63岁的妇女出现左颈腺病。实验室评估，包括全血细胞计数，完全新陈代谢，血清乳酸脱氢酶和β2-微球蛋白均正常。全身计算机断层扫描（CT）扫描显示diaphragm肌上方和下方弥漫性腺病。切除结节活检显示3A级结节性FL。 Ki67标记指数为40％至50％。骨髓活检显示小梁旁小梁CD20 +淋巴样聚集物。她接受了6个周期的苯达莫司汀（在第+1和+2天为90 mg / m2）和利妥昔单抗（在+2天为375 mg / m2），每个周期每4周进行一次。治疗结束后的后续CT扫描显示部分反应，腋窝腺病得以缓解，大腹膜后淋巴结明显缩小。 18个月后，她在没有B症状的情况下腹部绞痛。正电子发射断层扫描结合2-脱氧-2- [氟-18]氟-d-葡萄糖与CT（18F-FDG PET / CT）扫描显示广泛的腺病，弥漫性脾脏受累和实质性骨髓受累。活检2.4 cm右腋窝结节（SUVmax为16.1）显示受累于3A级FL，主要呈结节状生长。骨髓活检再次显示只有一小部分FL。从2015年5月开始，她接受了艾得拉西布（150 mg，每天两次）和利妥昔单抗（375 mg / m2，每月）。4个周期后，重复的CT扫描显示出完全的影像学反应。随后，Idelalisib因免疫介导的结肠炎而接受皮质类固醇治疗时被关押。一个月后，她以降低50％的剂量重新开始了艾达拉西布治疗。 2周后，她因双侧髋关节和腰部不适感到不适，但没有B症状而返回诊所。 2016年1月重新分期的18F-FDG PET / CT显示骨髓摄取显着。骨髓抽吸物显示出肿瘤细胞片，代表从具有淋巴细胞特征的中型细胞到具有多个核仁的非常大的非典型细胞的光谱。存在两种不同的组织学。一个保持与患者已知的具有主要结节型的FL相一致，另一个保持与HT相一致（大的非典型细胞表达PAX5，CD10，BCL2和c-MYC，而CD20，MPO，CD34，CD30和BCL6阴性） 。焦点区域显示出最佳的凝块切片上淡淡的，异质的末端脱氧核苷酸转移酶表达。 Ki67增殖指数高（4 + / 4）。荧光原位杂交分析显示2个具有MYC扩增和/或重排的群体，没有BCL6重排的迹象。核型分析表明，女性核型异常复杂，t（14; 18）且结构和数值异常多。她开始进行剂量调整的利妥昔单抗，依托泊苷，泼尼松，长春新碱，环磷酰胺和阿霉素，并同时进行预防

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