High-grade B cell lymphoma, unclassifiable, with blastoid features: an unusual morphological subgroup associated frequently with BCL2 and/or MYC gene rearrangements and a poor prognosis
High-grade B cell lymphoma, unclassifiable, with blastoid features: an unusual morphological subgroup associated frequently with BCL2 and/or MYC gene rearrangements and a poor prognosis
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DOI：
10.1111/j.1365-2559.2012.04301.x
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发表时间：
2012-11-01
期刊：
影响因子：
6.4
通讯作者：
Miranda, Roberto N.
中科院分区：
文献类型：
--
作者：
Kanagal-Shamanna, Rashmi;Medeiros, L. Jeffrey;Miranda, Roberto N.
目的：具有囊胚样特征的B细胞淋巴瘤亚群既不适合B淋巴母细胞淋巴瘤/白血病，也不适合囊胚样套细胞淋巴瘤。他们的分类是具有挑战性的，即使有完整的临床病理和遗传信息。在一次血液病研讨会上，专家们提出了高级别B细胞淋巴瘤这个术语，无法分类，具有囊胚特征，并建议进一步研究。方法和结果：我们描述了24例高级别B细胞淋巴瘤的临床病理，免疫表型和细胞遗传学结果，无法分类，具有囊胚特征。15例患者为新生，7例患者有淋巴瘤史。20例（83%）患者表现为淋巴结疾病。所有肿瘤均表达泛B细胞抗原，19个肿瘤中有17个（89%）具有生发中心B细胞免疫表型。荧光原位杂交（FISH）评估的16例肿瘤中有10例（63%）有MYC重排，18例中有13例（72%）携带high - bcl2, 15例中有9例（60%）两者都有（双重击中淋巴瘤）。中位总生存期为1.1年。根据2008年世界卫生组织的标准，15例被分类为B细胞淋巴瘤，无法分类，特征介于弥漫大B细胞淋巴瘤（DLBCL）和伯基特淋巴瘤之间，9例被分类为DLBCL，小的中心细胞变异。结论：高级别B细胞淋巴瘤，具有囊胚样特征，临床上具有侵袭性，生存率低。大多数肿瘤具有生发中心B细胞表型。MYC重排和IGH-BCL2是常见的，相似的60%是双重打击淋巴瘤。
Aims: A subset of B cell lymphomas with blastoid features do not fit either as B lymphoblastic lymphoma/leukaemia or blastoid mantle cell lymphoma. Their classification is challenging, even with complete clinicopathological and genetic information. At a haematopathology workshop, experts had suggested the term high-grade B cell lymphoma, unclassifiable, with blastoid features, and recommended further studies.Methods and results: We describe the clinicopathological, immunophenotypic and cytogenetic findings of 24 high-grade B cell lymphomas, unclassifiable, with blastoid features. Fifteen patients presented de novo and seven patients had a history of lymphoma. Twenty patients (83%) presented with nodal disease. All tumours expressed pan-B cell antigens and 17 (89%) of 19 tumours assessed had a germinal centre B cell immunophenotype. Ten (63%) of 16 tumours assessed by fluorescence in-situ hybridization (FISH) had MYC rearrangement, 13 of 18 (72%) carried IGH-BCL2 and nine of 15 (60%) had both (double-hit lymphoma). The median overall survival was 1.1 years. Using 2008 World Health Organization criteria, 15 cases were classified as B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma (DLBCL) and Burkitt lymphoma, and nine as DLBCL, small centroblastic variant.Conclusion: High-grade B cell lymphomas, unclassifiable, with blastoid features are clinically aggressive with poor survival. Most neoplasms have a germinal centre B cell phenotype. MYC rearrangements and IGH-BCL2 are common, and similar to 60% are double-hit lymphomas.