Sevilla Deborah W, Gong Jerald Z, Goodman Barbara K, Buckley Patrick J, Rosoff Philip, Gockerman Jon P, Lagoo Anand S
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Am J Clin Pathol. 2007 Dec;128(6):981-91. doi: 10.1309/C1PP15P7CDB8UY39.
In the World Health Organization classification, cases with classical Burkitt morphologic features and a very high proliferation fraction but without the MYC translocation are not clearly designated as a separate entity and are usually categorized as diffuse large B-cell lymphoma (DLBCL). We identified from our records 33 cases of highly aggressive mature B-cell neoplasms from 8 children and 25 adults with typical Burkitt cytomorphologic, histologic, and immunophenotypic (CD20+/CD10+ and surface immunoglobulin-positive) features. Rearrangement of MYC (MYC+) was present in only 18 of 33 cases, but the proliferation fraction was more than 90% in all MYC-cases (no MYC rearrangement). The immunophenotype of the lymphoma cells in the 2 groups was similar. Although children with MYC+ and MYC- neoplasms were treated with chemotherapy regimens appropriate for Burkitt lymphoma, adults with MYC- lymphomas received less aggressive therapy usually given for DLBCL. Survival analysis showed that adults in the MYC- group had an inferior outcome compared with adults with MYC+ disease. Provisional identification of MYC- lymphomas with typical Burkitt morphologic features as an entity separate from DLBCL will facilitate further studies and possible categorization as a separate entity.
在世界卫生组织的分类中，具有经典伯基特形态学特征且增殖分数非常高但无MYC易位的病例未被明确指定为一个单独的实体，通常被归类为弥漫性大B细胞淋巴瘤（DLBCL）。我们从记录中识别出33例高度侵袭性成熟B细胞肿瘤病例，来自8名儿童和25名成人，具有典型的伯基特细胞形态学、组织学和免疫表型（CD20+/CD10+且表面免疫球蛋白阳性）特征。33例中仅18例存在MYC重排（MYC+），但所有MYC阴性病例（无MYC重排）的增殖分数均超过90%。两组淋巴瘤细胞的免疫表型相似。虽然MYC阳性和MYC阴性肿瘤的儿童接受了适合伯基特淋巴瘤的化疗方案，但MYC阴性淋巴瘤的成人接受的通常是用于DLBCL的侵袭性较小的治疗。生存分析表明，与MYC阳性疾病的成人相比，MYC阴性组的成人预后较差。将具有典型伯基特形态学特征的MYC阴性淋巴瘤临时识别为一个与DLBCL不同的实体，将有助于进一步研究，并可能将其归类为一个单独的实体。
