Original Article
Clinicopathological and molecular genetic features of Burkitt-like lymphoma with 11q aberration
Zhang Yanping, Zhang Lan, Zhang Dandan, Wang Guannan, Zhao Wugan, Jian Xiangyu, Li Wencai
Published 2021-06-08
Cite as Chin J Pathol, 2021, 50(6): 604-608. DOI: 10.3760/cma.j.cn112151-20210204-00127
Abstract
ObjectiveTo investigate the clinicopathological features, molecular genetics, treatment and prognosis of Burkitt-like lymphoma with 11q aberration (BLL-11q).
MethodsSix cases of BLL-11q diagnosed at the First Affiliated Hospital of Zhengzhou University, from January 2016 to January 2020 were reviewed and analyzed using hematoxylin-eosin staining, immunohistochemistry, EBER in situ hybridization and fluorescence in situ hybridization. Clinical information including follow-up data was collected and analyzed.
ResultsThe median age of the six immunocompetent patients was 29 years (range 20-38 years) and the male to female ratio was 5∶1. All patients had nodal disease in the head and neck region. Five patients had Ann Arbor stage Ⅰ-Ⅱ disease, while one patient had stage Ⅳ disease. Lymph nodes showed partial or total architectural effacement by a diffuse proliferation of monomorphic lymphocytes. Four cases were morphologically similar to Burkitt lymphoma, and two cases were unclassified with histological features between Burkitt lymphoma and diffuse large B-cell lymphoma. Mitotic figures, apoptosis and necrosis were conspicuous. Five cases exhibited the"starry sky"pattern. CD20, CD10 and bcl-6 were diffusely and strongly positive. The Ki-67 index was more than 95%. The follicular-dendritic-cell meshwork was noted in one case using CD21 stain. C-MYC was expressed variably. CD3, bcl-2, MUM-1, CD30 and TDT were negative in all cases. EBER in situ hybridization was also all negative. FISH analyses using C-MYC, bcl-2 and bcl-6 break-apart probes were all negative. All cases had the 11q23.3 gain/11q24.3 loss pattern, and 11q23.3 amplification was found in one case. IgH and IRF4 break-apart probes analysis was also negative. All patients were alive with no disease after a follow-up of 4 to 19 months.
ConclusionBLL-11q is a rare lymphoma that resembles Burkitt lymphoma morphologically and phenotypically, but lacks C-MYC gene rearrangements. Instead, it has a chromosome-11q alteration characterized by proximal gains and telomeric losses. It′s necessary to improve our understanding of BLL-11q to avoid misdiagnosis and missed diagnosis.
Key words:
Lymphoma; Pathology, molecular; Diagnosis, differential
Contributor Information
Zhang Yanping
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Zhang Lan
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Zhang Dandan
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Wang Guannan
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Zhao Wugan
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Jian Xiangyu
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
Li Wencai
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052
