Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Histopathology. 2012 Nov;61(5):945-54. doi: 10.1111/j.1365-2559.2012.04301.x. Epub 2012 Jul 17.
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.
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.
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 ~60% are double-hit lymphomas.
具有母细胞样特征的 B 细胞淋巴瘤亚类既不符合 B 淋巴母细胞性淋巴瘤/白血病，也不符合母细胞样套细胞淋巴瘤。即使有完整的临床病理和遗传学信息，其分类也具有挑战性。在血液病理学研讨会上，专家们建议使用“高级别 B 细胞淋巴瘤，分类不明，具有母细胞样特征”这一术语，并建议进一步研究。
我们描述了 24 例高级别 B 细胞淋巴瘤，分类不明，具有母细胞样特征的临床病理、免疫表型和细胞遗传学发现。15 例患者为初发，7 例患者有淋巴瘤病史。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 例评估的肿瘤中有 17 例（89%）具有生发中心 B 细胞免疫表型。16 例评估的肿瘤中有 10 例（63%）通过荧光原位杂交（FISH）存在 MYC 重排，18 例中有 13 例（72%）存在 IGH-BCL2，15 例中有 9 例（60%）同时存在（双打击淋巴瘤）。总生存中位数为 1.1 年。根据 2008 年世界卫生组织标准，15 例病例被分类为高级别 B 细胞淋巴瘤，分类不明，介于弥漫性大 B 细胞淋巴瘤（DLBCL）和伯基特淋巴瘤之间，9 例为 DLBCL，小中心母细胞变异型。
高级别 B 细胞淋巴瘤，分类不明，具有母细胞样特征，临床侵袭性强，生存预后差。大多数肿瘤具有生发中心 B 细胞表型。MYC 重排和 IGH-BCL2 很常见，约 60%为双打击淋巴瘤。
