Burkitt-like lymphoma with 11q aberration mimicking appendicitis in a young man — a diagnostic and therapeutic challenge
, 2, 2, 1
Abstract
Burkitt-like lymphoma with 11q aberration (BLL-11q) is a rare entity affecting mainly young people and generally manifesting with nodal involvements. The lack of MYC translocation is the hallmark of BLL-11q while the morphological features of BLL-11q resemble those of classical Burkitt lymphoma (BL). Therefore, BLL-11q is usually treated according to the recommendations for classical BL. However, there is no standard of care in patients with BLL‑11q. This research presents a 19-year-old man with the initial diagnosis of appendicitis. Microscopic examination of resected tissue, supported by immunohistochemistry and fluorescence in situ hybridization finally confirmed the diagnosis of BLL-11q. The patient was treated with R‑hyper-CVAD (rituximab, cyclophosphamide, vincristine, doxorubicin and dexamethasone alternately with rituximab, methotrexate, cytarabine), a protocol dedicated to classical BL. After 3 cycles of that treatment, complete metabolic response was achieved. Due to the prolonged myelosuppression observed after 4 cycles of the immunochemotherapy, the treatment was de-escalated to 2 additional cycles of R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, prednisolone). Post-therapeutic neutropenia grades 3 and 4 without any infections were observed up to six months after the end of treatment. The patient displayed no clinical or laboratory symptoms of the disease during the 18-months follow-up.
Keywords: Burkitt-like lymphoma with 11q aberrationsymptomstreatment
References
- Swerdlow SH, Campo E, Harris NL. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th edition. IARC, Lyon, 2017: 334.
- Love C, Sun Z, Jima D, et al. The genetic landscape of mutations in Burkitt lymphoma. Nat Genet. 2012; 44(12): 1321–1325.
- Rohde M, Bonn BR, Zimmermann M, et al. MMML-MYC-SYS Project, ICGC MMML-Seq Project. Relevance of ID3-TCF3-CCND3 pathway mutations in pediatric aggressive B-cell lymphoma treated according to the non-Hodgkin Lymphoma Berlin-Frankfurt-Münster protocols. Haematologica. 2017; 102(6): 1091–1098.
- Thomas DA, Faderl S, O'Brien S, et al. Chemoimmunotherapy with hyper-CVAD plus rituximab for the treatment of adult Burkitt and Burkitt-type lymphoma or acute lymphoblastic leukemia. Cancer. 2006; 106(7): 1569–1580.
- U.S. Department of Health and Human Services, National Cancer National, Institutes of Health Institute. Common Terminology Criteria for Adverse Events (CTCAE), Version 5.0. https://ctep.cancer.gov/protocoldevelopment/electronic_applications/docs/ctcae_v5_quick_reference_5x7.pdf (November 27, 2017).
- Rymkiewicz G, Grygalewicz B, Chechlinska M, et al. A comprehensive flow-cytometry-based immunophenotypic characterization of Burkitt-like lymphoma with 11q aberration. Mod Pathol. 2018; 31(5): 732–743.
- Salaverria I, Martin-Guerrero I, Wagener R, et al. Molecular Mechanisms in Malignant Lymphoma Network Project, Berlin-Frankfurt-Münster Non-Hodgkin Lymphoma Group. A recurrent 11q aberration pattern characterizes a subset of MYC-negative high-grade B-cell lymphomas resembling Burkitt lymphoma. Blood. 2014; 123(8): 1187–1198.
- Havelange V, Ameye G, Théate I, et al. The peculiar 11q-gain/loss aberration reported in a subset of MYC-negative high-grade B-cell lymphomas can also occur in a MYC-rearranged lymphoma. Cancer Genet. 2016; 209(3): 117–118.
- Grygalewicz B, Woroniecka R, Rymkiewicz G, et al. The 11q-gain/loss aberration occurs recurrently in MYC-negative Burkitt-like lymphoma with 11q aberration, as well as MYC-positive burkitt lymphoma and MYC-positive high-grade B-cell lymphoma, NOS. Am J Clin Pathol. 2017; 149(1): 17–28.
- Gonzalez-Farre B, Ramis-Zaldivar JE, Salmeron-Villalobos J, et al. Burkitt-like lymphoma with 11q aberration: a germinal center-derived lymphoma genetically unrelated to Burkitt lymphoma. Haematologica. 2019; 104(9): 1822–1829.
- Wang J, Ma Li, Guo J, et al. Burkitt-like lymphoma with 11q aberration in a patient with AIDS and a patient without AIDS: Two cases reports and literature review. Open Med (Wars). 2021; 16(1): 428–434.
- Moshref Razavi H, Hrynchak M. Unusual presentation of Burkitt-like lymphoma with 11q aberration in an elderly patient. Blood. 2019; 133(4): 381.
- Ferreiro JF, Morscio J, Dierickx D, et al. Post-transplant molecularly defined Burkitt lymphomas are frequently MYC-negative and characterized by the 11q-gain/loss pattern. Haematologica. 2015; 100(7): e275–e279.
