Kodet R, Mrhalová M, Stejskalová E, Kabícková E
Department of Pathology and Molecular Medicine, Charles University, 2nd Faculty of Medicine and Faculty Hospital Motol, Prague, Czech Republic.
Cesk Patol. 2011 Jul;47(3):106-14.
Burkitt lymphoma (BL) is a well characterized entity. For atypical findings a term Burkitt-like lymphoma (B-LL) was applied in the past, but the interpretation of the morphological appearances was subjective and poorly reproducible. We used a combined approach (morphology using classical histological staining; immunohistochemistry-IHC; fluorescence in situ hybridization-FISH on interphase nuclei; cytogenetics) to perform a retrospective study on 39 patients diagnosed as BL and B-LL at our department in the years 1982 to 2002. By FISH we demonstrated t(8;14)(q24;q32) in 31 patients; in further two we found a break at 8q24, suggestive of a variant translocation. In three patients with the cytogenetic investigation available we confirmed the findings of FISH--two lymphomas had the t(8;14)(q24;q32), one had t(2;8)(p12;q24). IHC showed CD20, CD10, BCL-6, p53 expression, and Ki-67 antigen in > 95% of the tumor cell population in a majority of the patients. There was a group of 4 patients in whom the t(8;14)(q24;q32) or a break at 8q24 were not found (FISH). These cases were reclassified within the WHO defined grey zone subgroup of B-cell lymphoma unclassifiable with features intermediate between diffuse large cell lymphoma (DLBCL) and Burkitt lymphoma--I-DLBCL/BL. Two further cases were reclassified as DLBCL based on a combined IHC and FISH findings. A lymphoma of one of these patients had breaks at 3q27 (BCL6) and at 14q32 (IGH) suggestive of t(3;14)(q27;q32). The overall survival estimate of 33 patients with the diagnosis of BL was 54%. Most of deaths occurred within 6 months after the tumor diagnosis. The unfavorable clinical outcome appears to be associated with a strong expression of the p53 protein in the tumor cell population. Individually utilized methods in the diagnosis of BL may lead to false diagnostic conclusions. A combined approach helps to establish a more reliable diagnosis of BL and to separate grey zone lymphomas I-DLBCL/BL and DLBCL with morphological mimics of BL to start adequate treatment. I-DLBCL/BL is a non-homogenous group of lymphomas necessitating further analysis in a prospective study.
伯基特淋巴瘤（BL）是一种特征明确的疾病实体。过去，对于非典型表现的病例使用了“伯基特样淋巴瘤（B-LL）”这一术语，但对其形态学表现的解读主观且重复性差。我们采用了一种综合方法（使用经典组织学染色的形态学、免疫组织化学 - IHC、间期核荧光原位杂交 - FISH、细胞遗传学）对1982年至2002年间在我科诊断为BL和B-LL的39例患者进行回顾性研究。通过FISH，我们在31例患者中发现了t(8;14)(q24;q32)；在另外2例中，我们在8q24处发现了断裂，提示存在变异易位。在有细胞遗传学检查结果的3例患者中，我们证实了FISH的结果——2例淋巴瘤有t(8;14)(q24;q32)，1例有t(2;8)(p12;q24)。免疫组化显示，大多数患者中超过95%的肿瘤细胞群体表达CD20、CD10、BCL-6、p53以及Ki-67抗原。有一组4例患者未发现t(8;14)(q24;q32)或8q24处的断裂（FISH）。这些病例被重新分类为世界卫生组织定义的B细胞淋巴瘤灰色区亚组，其特征介于弥漫性大B细胞淋巴瘤（DLBCL）和伯基特淋巴瘤之间——I-DLBCL/BL。另外2例基于免疫组化和FISH的联合结果被重新分类为DLBCL。其中1例患者的淋巴瘤在3q27（BCL6）和14q32（IGH）处有断裂，提示t(3;14)(q27;q32)。33例诊断为BL的患者的总生存估计为54%。大多数死亡发生在肿瘤诊断后的6个月内。不良的临床结局似乎与肿瘤细胞群体中p53蛋白的强表达有关。单独使用的BL诊断方法可能会导致错误的诊断结论。综合方法有助于更可靠地诊断BL，并区分灰色区淋巴瘤I-DLBCL/BL以及具有BL形态学模仿特征的DLBCL，从而开始适当的治疗。I-DLBCL/BL是一组异质性淋巴瘤，需要在前瞻性研究中进一步分析。
