Page 144 - 2019_09-HaematologicaMondo-web

Ferrata Storti Foundation
Haematologica 2019 Volume 104(9):1822-1829
Non-Hodgkin Lymphoma
Burkitt-like lymphoma with 11q aberration: a germinal center-derived lymphoma genetically unrelated to Burkitt lymphoma
Blanca Gonzalez-Farre,1,2,3* Joan Enric Ramis-Zaldivar,2,3* Julia Salmeron- Villalobos,2 Olga Balagué,1,2,3 Verónica Celis,4 Jaime Verdu-Amoros,5 Ferran Nadeu,2,3 Constantino Sábado,6 Antonio Ferrández,7 Marta Garrido,8 Federico García-Bragado,9 María Dolores de la Maya,10 José Manuel Vagace,10 Carlos Manuel Panizo,11 Itziar Astigarraga,12 Mara Andrés,13 Elaine S. Jaffe,14 Elias Campo1,2,3* and Itziar Salaverria2,3*
1Hematopathology Unit, Hospital Clínic de Barcelona, University of Barcelona, Barcelona, Spain; 2Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; 3Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain; 4Pediatric Oncology Department, Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain; 5Pediatric Oncology Department, Hospital Clínico Universitario de Valencia, Valencia, Spain; 6Pediatric Oncology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain; 7Pathology Department, Hospital Clínico de Valencia, Valencia, Spain; 8Pathology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain; 9Pathology Department, Complejo Hospitalario de Navarra, Pamplona, Spain; 10Pediatric Hematology Department, Hospital Materno Infantil de Badajoz, Badajoz, Spain; 11Department of Hematology, Clínica Universidad de Navarra and Instituto de Investigación Sanitaria de Navarra (IdiSNA), Pamplona, Spain; 12Pediatrics Department, Hospital Universitario Cruces, IIS Biocruces Bizkaia, UPV/EHU, Barakaldo, Spain; 13Pediatric Oncology Department, Hospital La Fe, Valencia, Spain and 14Hematopathology Section, Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
*BGF, JERZ, EC and IS contributed equally to this work.
ABSTRACT
Burkitt-like lymphoma with 11q aberration is characterized by patho- logical features and gene expression profile resembling those of Burkitt lymphoma but lacks the MYC rearrangement and carries an 11q-arm aberration with proximal gains and telomeric losses. Whether this lymphoma is a distinct category or a particular variant of other recognized entities is controversial. To improve the understanding of Burkitt-like lym- phoma with 11q aberration we performed an analysis of copy number alterations and targeted sequencing of a large panel of B-cell lymphoma- related genes in 11 cases. Most patients had localized nodal disease and a favorable outcome after therapy. Histologically, they were high grade B-cell lymphoma, not otherwise specified (8 cases), diffuse large B-cell lymphoma (2 cases) and only one was considered as atypical Burkitt lymphoma. All cases had a germinal center B-cell signature and phenotype with frequent LMO2 expression. The patients with Burkitt-like lymphoma with 11q aber- ration had frequent gains of 12q12-q21.1 and losses of 6q12.1-q21, and lacked common Burkitt lymphoma or diffuse large B-cell lymphoma alter- ations. Potential driver mutations were found in 27 genes, particularly involving BTG2, DDX3X, ETS1, EP300, and GNA13. However, ID3, TCF3, or CCND3 mutations were absent in all cases. These results suggest that Burkitt-like lymphoma with 11q aberration is a germinal center-derived lymphoma closer to high-grade B-cell lymphoma or diffuse large B-cell lym- phoma than to Burkitt lymphoma.
Introduction
Our knowledge of lymphomas in children and young adults has increased dramat- ically in the last years with the identification of several subtypes that predominantly occur in this age subgroup.1-4 One of these recently recognized categories is Burkitt- like lymphoma with 11q aberration (BLL-11q) which has morphological, phenotyp-
Correspondence:
ITZIAR SALAVERRIA
isalaver@clinic.cat
Received: October 5, 2018. Accepted: February 7, 2019. Pre-published: February 7, 2019.
doi:10.3324/haematol.2018.207928
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/104/9/1822
©2019 Ferrata Storti Foundation
Material published in Haematologica is covered by copyright. All rights are reserved to the Ferrata Storti Foundation. Use of published material is allowed under the following terms and conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode. Copies of published material are allowed for personal or inter- nal use. Sharing published material for non-commercial pur- poses is subject to the following conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode, sect. 3. Reproducing and sharing published material for com- mercial purposes is not allowed without permission in writing from the publisher.
1822
haematologica | 2019; 104(9)
ARTICLE