Pediatric high-grade B-cell lymphoma with 11q aberrations presenting with hematemesis and epistaxis

Pediatric high-grade B-cell lymphoma with 11q aberrations presenting with hematemesis and epistaxis | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed. Data may be preliminary. 22 December 2025 V1 Latest version Share on Pediatric high-grade B-cell lymphoma with 11q aberrations presenting with hematemesis and epistaxis Authors : Yingying Li 0009-0007-4044-0317 [email protected] , Yan nan Xu , Shao wei Gu , Ren xu Yu , and Xuezhong Li Authors Info & Affiliations https://doi.org/10.22541/au.176640691.12877150/v1 155 views 81 downloads Contents Abstract Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract Background：High-grade B-cell lymphoma with 11q aberrations (HGBCL-11q) is a newly defined entity in the 2022 WHO classification, rarely reported in pediatric nasopharyngeal cases. Case Presentation:We describe an unusual case of a 7-year-old boy who initially exhibited symptoms of hematemesis and epistaxis, subsequently caused anemia. The imaging studies revealed an oropharyngeal mass Title: Pediatric high-grade B-cell lymphoma with 11q aberrations presenting with hematemesis and epistaxis Yingying Li a , PhD, Yannan Xu a , MD, Shaowei Gu b , MPH, Renxu Yu c , MPH, Xuezhong Li b,* , PhD a Department of Otorhinolaryngology, Yantai Affiliated Hospital of Binzhou Medical University, The Second School of Clinical Medicine of Binzhou Medical University, Yantai, China b Department of Otorhinolaryngology, Qilu Hospital of Shandong University, National Health Commission Key Laboratory of Otorhinolaryngology ( Shandong University), Jinan, China c Department of Ultrasonic, Qilu Hospital of Shandong University, Jinan,China * Department of Otorhinolaryngology, Qilu Hospital of Shandong University, National Health Commission Key Laboratory of Otorhinolaryngology ( Shandong University), Jinan, China. E-mail address: [email protected] (X. Li) Short Ru nning Title: Pediatric HGBCL-11q with Bleeding Source of Financial Support: None. Institution where the work was done: Binzhou Medical University Yantai Affiliated Hospital, Yantai, Shandong, China Conflict of Interest Statement: The authors have no conflicts of interest to disclose. Corresponding Author (correspondence, reprint requests, and proofs): Yingying Li, PhD Department of Otorhinolaryngology, Yantai Affiliated Hospital of Binzhou Medical University, The Second School of Clinical Medicine of Binzhou Medical University, Yantai, China Email: [email protected] Phone: +86-15866494017 Abstract High-grade B-cell lymphoma with 11q aberrations (HGBCL-11q) is a newly defined entity in the 2022 WHO classification, rarely reported in pediatric nasopharyngeal cases.We describe an unusual case of a 7-year-old boy who initially exhibited symptoms of hematemesis and epistaxis, subsequently caused anemia. The imaging studies revealed an oropharyngeal mass situated at the adenoid glands. Subsequent histological analysis and FISH testing definitively confirmed the diagnosis of high-grade B-cell lymphoma with 11q aberrations (HG/LBCL-11q).This case highlights the importance of molecular testing (FISH for 11q) in pediatric lymphomas with atypical bleeding symptoms, preventing misdiagnosis as benign adenoid hypertrophy. Key words: Pediatric; Nasopharyngeal neoplasm; Nasal endoscopy; High-grade/large B-cell lymphoma(HG/LBCL); Hematemesis Key Clinical Message For pediatric patients presenting with atypical bleeding symptoms like hematemesis and epistaxis alongside a nasopharyngeal mass, high-grade B-cell lymphoma with 11q aberrations (HGBCL-11q) should be considered in differential diagnosis to avoid misdiagnosis as benign adenoid hypertrophy. Introduction Lymphoma, the third most prevalent cancer, is divided into Non-Hodgkin’s (NHL) and Hodgkin’s Lymphoma (HL) [1]. In NHL, Waldeyer’s ring is the most common extranodal site affected in the head and neck [2]. The high-grade/large B-cell lymphoma with 11q aberration (HG/LBCL-11q), resembling Burkitt lymphoma but without MYC rearrangement, is classified as a novel entity in the 2022 ICC and WHO 5th edition [3]. Nasopharyngeal NHL (NNHL) symptoms include nasal obstruction, bleeding, discharge, crusting, hyponasal voice, tinnitus, ear pain, and effusion [4]. HG/LBCL-11q in the nasopharynx is rare, with a low incidence of specific genetic abnormalities. Case report A 7-year-old child presented to the emergency department in May 2024 with complaints of hematemesis and epistaxis. Blood tests revealed anemia (Hb 64g/L), prompting admission to the pediatric ICU. In the pediatric ICU, the child’s urinalysis, stool exam, and gastrointestinal endoscopy were negative. Bone marrow aspiration was normal. Past visits showed ENT clinic complaint of nasal congestion and nasal bleeding, diagnosed as adenoid hypertrophy via nasal endoscopy. The patient was scheduled for a nasal endoscopic biopsy and debulking of the nasopharyngeal mass, assisted by transnasal coblation under general anesthesia (GA). The nasal congestion symptoms were significantly relieved. The immunophenotype of the pathological results was consistent with Burkitt lymphoma (high Ki-67, CD10+, BCL2-). As a prudent measure, FISH testing was performed, which revealed no MYC/BCL2/BCL6 rearrangements, ruling out double-/triple-hit lymphoma. This prompted FISH analysis of the 11q region, leading to a confirmed diagnosis of HGBL-11q. After diagnosis, the patient was treated with the R3 chemotherapy regimen, which consists of lenalidomide plus dose-adjusted R-EPOCH (DA-EPOCH-R). A follow-up MRI in May 2025 demonstrated smooth nasopharyngeal soft tissue with no obvious mass. MRI showed a 5.1x2.2 cm mass in the nasopharyngeal cavity, pharyngeal recesses obscured, and nasopharyngeal cavity deformed with bilateral asymmetry. T1WI/T2WI showed iso-signals to muscle, with high DWI and low ADC signals, indicating significant diffusion restriction. Ultrasound revealed multiple enlarged, hypoechoic neck lymph nodes bilaterally, with unclear boundaries, thickened cortex, and disorganized blood flow. The largest lymph nodes measured 2.7x1.7cm on the left (II region) and 2.1x1.0cm on the right (I region). Nasopharyngeal mass debulking was done with an adenotome. Prior to the operation, nasal endoscopy showed a large mass occupying 95% of the nasopharynx, nearly blocking the airway. During the procedure, the lesion surface was inflamed with necrosis. Biopsy showed B-cell lymphoma, possibly Burkitt. IHC: CD20+, CD79a+, CD19+, PAX-5+, CD10+, c-Myc 60%, Bcl-6 80%, CD21 (FDC network positive), P53 (+, 5%), and the Ki-67 (+,67%-90%). FISH confirmed high-grade B-cell lymphoma with 11q abnormalities. FISH results: 11q24.3+, 11q23.3+; Bcl-2-, Bcl-6-, C-myc-. PET-CT showed an irregular nasopharyngeal mass extending into the nasal/oropharynx (7.4x3.6cm), SUVmax 26.9. Enlarged lymph nodes in neck II-III with high FDG, largest 2.7x2.1cm, SUVmax 27.2. High FDG uptake was also noted in the sphenoid bone, S1 vertebra, and right ischium, SUVmax 14.0. Discussion There is a tendency for it to be overlooked and misdiagnosed when pediatric patients are being treated. So when a 7-year-old boy, who was not a high-risk demographic, presented with hematemesis and epistaxis as his first clinical features, it should be considered more comprehensively, especially when presented with a nasopharyngeal mass. The diagnosis of lymphoma requires integrating clinical manifestations with physical, laboratory, imaging, and pathological examinations. At the 2022 EA4HP/SH lymphoma workshop, it was recommended to utilize clinical manifestations, physical examination, laboratory examination, imaging examinations (including CT, MRI, PET-CT, Ultrasound), pathological examination, and FISH for diagnosing the disease [5]. HG/LBCL-11q lymphoma is rare, especially in pediatric patients. Besides being a newly discovered subtype of lymphoma, it is characterized by unique chromosomal aberrations and clinical features, and shares morphological similarities with BL. The crucial difference lies in its absence of MYC rearrangement, which is a hallmark feature of BL but absent in HG/LBCL-11q. For patients with lymphomas exhibiting HG features but without MYC translocation, it is recommended to perform FISH testing for 11q aberrations to confirm the presence of HG/LBCL-11q. This testing aids in providing a more precise basis for pathological diagnosis. As the disease is diagnosed more accurately, we can enable the provision of more targeted treatment plans for patients. By the date of submission, the patient had completed chemotherapy and had no recurrence. Disclosure statement All authors have nothing relevant to disclose. Ethical statement This study obtained approval from the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University. Written informed consent for the publication of the patient’s clinical details was obtained from the patient. References [1] Storck K, Brandstetter M, Keller U, Knopf A. Clinical presentation and characteristics of lymphoma in the head and neck region. Head & Face Medicine 2019;15:1-8. https://doi.org/10.1186/s13005-018-0186-0 [2] Han AY, Kuan EC, Alonso JE, Badran KW, St. John MA. Epidemiology of nasopharyngeal lymphoma in the United States: a population-based analysis of 1119 cases. Otolaryngology–Head and Neck Surgery 2017;156(5):870-6. https://doi.org/10.1177/0194599817695808 [3] Salaverria I, Martin-Guerrero I, Wagener R, et al. A recurrent 11q aberration pattern characterizes a subset of MYC-negative high-grade B-cell lymphomas resembling Burkitt lymphoma. Blood, The Journal of the American Society of Hematology, 2014, 123(8): 1187-1198. https://doi.org/10.1182/blood-2013-06-507996 [4] Kwong Y L, CHAN, Liang R, et al. CD56+ NK lymphomas: clinicopathological features and prognosis. British Journal of Haematology, 1997, 97(4): 821-829. https://doi.org/10.1046/j.1365-2141.1997.1462962.x [5] Quintanilla-Martinez L, Laurent C, Soma L, et al. Emerging entities: high-grade/large B-cell lymphoma with 11q aberration, large B-cell lymphoma with IRF4 rearrangement, and new molecular subgroups in large B-cell lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop. Virchows Archiv, 2023, 483(3): 281-298. https://doi.org/10.1007/s00428-023-03590-x Figure Legends Figure 1. A, B: MRI images showing a 5.1x2.2 cm mass in the nasopharyngeal cavity (arrows), with obscured pharyngeal recesses and bilateral asymmetry. C, D: Diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) map demonstrating high DWI and low ADC signals within the mass, indicating significant diffusion restriction. E, F: Ultrasound images showing enlarged, hypoechoic lymph nodes in the left level II region (E) and right level I region (F). G: Nasal endoscopic view prior to surgery, showing a large mass occupying approximately 95% of the nasopharyngeal space. H: Intraoperative view of the necrotic and inflamed surface of the mass during debulking. I, J: Photomicrographs of the biopsy specimen (I: H&E stain; J: Immunohistochemistry for Ki-67, showing a high proliferative index of 67-90%). K: Fluorescence in situ hybridization (FISH) image confirming 11q aberrations (11q24.3 and 11q23.3 gains). L: Maximum intensity projection (MIP) PET-CT image showing hypermetabolic activity in the nasopharyngeal mass, cervical lymph nodes, and multiple skeletal sites. Information & Authors Information Version history V1 Version 1 22 December 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Keywords and throat ear hematology nose oncology Authors Affiliations Yingying Li 0009-0007-4044-0317 [email protected] Yantai Affiliated Hospital of Binzhou Medical University View all articles by this author Yan nan Xu Yantai Affiliated Hospital of Binzhou Medical University View all articles by this author Shao wei Gu Qilu Hospital of Shandong University View all articles by this author Ren xu Yu Qilu Hospital of Shandong University View all articles by this author Xuezhong Li Qilu Hospital of Shandong University View all articles by this author Metrics & Citations Metrics Article Usage 155 views 81 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Yingying Li, Yan nan Xu, Shao wei Gu, et al. Pediatric high-grade B-cell lymphoma with 11q aberrations presenting with hematemesis and epistaxis. Authorea . 22 December 2025. 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