Vol 13, No 2 (2022)
Case report
Published online: 2022-05-21

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Burkitt-like lymphoma with 11q aberration mimicking appendicitis in a young man — a diagnostic and therapeutic challenge

Ryszard Swoboda1, Karolina Gajda2, Roman Lamch2, Sebastian Giebel1
Hematology in Clinical Practice 2022;13(2):75-79.

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.

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Hematology in Clinical Practice