Glazyrin Alexey, Patel Chirag, Kujtan Lara, Madhusudhana Sheshadri
Department of Pathology, Truman Medical Center, University of Missouri at Kansas City, Kansas City, Missouri, USA.
Department of Internal Medicine - Hematology/Oncology, Truman Medical Center, University of Missouri at Kansas City, Kansas City, Missouri, USA.
Acta Haematol. 2018;139(2):84-88. doi: 10.1159/000486360. Epub 2018 Feb 6.
Low-grade follicular lymphomas are genetically characterized by the translocation t(14; 18)(q32;q21) with BCL2 gene rearrangements. Marginal zone lymphomas are often associated with translocations or transcriptional deregulations of the MALT gene. We report 2 cases of lymphomas which harbor both the t(14;18)(q32;q21) translocation and MALT gene upregulation. Patients presented with numerous circulating atypical lymphocytes. Lymph node biopsy in both cases on HE staining demonstrated vague nodularity readily highlighted by CD10, CD23, or BCL6. Staining with CD20 and BCL2 demonstrated monotonous diffuse effacement of normal architecture with tumor cells without obvious follicular structures. Morphologically, tumor cells were consistent with centrocytes. Bone marrow biopsy demonstrated a combined peritrabecular and interstitial distribution of the tumor cells. These cases present substantial difficulties for diagnosis and classification. Clinical and morphological features were mostly consistent with follicular lymphoma, with a few features more often seen in marginal zone lymphomas (leukemic presentation, no CD10 in circulating cells, interstitial location of tumor cells in bone marrow); therefore, these cases were finally classified as follicular lymphoma grade I. Both patients were treated with standard chemotherapy regimens for follicular and nongastric MALT lymphomas with a good response to date.
低级滤泡性淋巴瘤的基因特征是存在t(14; 18)(q32;q21)易位及BCL2基因重排。边缘区淋巴瘤常与MALT基因的易位或转录失调相关。我们报告2例淋巴瘤病例,其同时存在t(14;18)(q32;q21)易位和MALT基因上调。患者出现大量循环中的非典型淋巴细胞。两例病例的淋巴结活检HE染色显示模糊的结节状,CD10、CD23或BCL6可使其易于凸显。CD20和BCL2染色显示肿瘤细胞使正常结构呈单调弥漫性消失,无明显滤泡结构。形态学上,肿瘤细胞与中心细胞一致。骨髓活检显示肿瘤细胞呈小梁周围和间质混合分布。这些病例在诊断和分类上存在很大困难。临床和形态学特征大多与滤泡性淋巴瘤一致,但有一些特征更常见于边缘区淋巴瘤(白血病表现、循环细胞中无CD10、骨髓中肿瘤细胞位于间质);因此,这些病例最终被分类为I级滤泡性淋巴瘤。两名患者均接受了针对滤泡性和非胃MALT淋巴瘤的标准化化疗方案治疗,至今反应良好。