Successful CNS-Centric Therapeutic Management and Genomic Profiling of Primary Cranial Vault Diffuse Large B-Cell Lymphoma.

IF 2.1 Q3 HEMATOLOGY Journal of Blood Medicine Pub Date : 2023-01-01 DOI:10.2147/JBM.S391094
Jordan Davis, ErinMarie O Kimbrough, Muhamad Alhaj Moustafa, Liuyan Jiang, Vivek Gupta, Ephraim Parent, Han W Tun
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Abstract

Primary cranial vault lymphoma (PCVL) is a rare lymphoma involving the skull with or without extra- and intracranial extension. Most cases of PCVL are diffuse large B-cell lymphoma (DLBCL). We report a case of primary cranial vault diffuse large B-cell lymphoma (PCV-DLBCL) that was successfully treated with anthracycline-based chemoimmunotherapy (CIT) alternating with central nervous system (CNS)-directed CIT with high-dose methotrexate and high-dose cytarabine. CNS-centric therapy was given for suspected cerebral cortical involvement and presumed elevated risk of CNS recurrence. The patient has remained in complete remission for 4.25 years following treatment. We suggest that PCV-DLBCL is potentially curable with CNS-directed therapy. Additionally, we provide genomic profiling results indicating an indeterminate cell of origin and multiple genetic mutations which are not frequently seen in DLBCL.

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原发性颅穹窿弥漫性大b细胞淋巴瘤以中枢神经系统为中心的成功治疗管理和基因组分析。
原发性颅穹窿淋巴瘤(PCVL)是一种罕见的累及颅骨的淋巴瘤,伴或不伴颅外及颅内延伸。PCVL多为弥漫性大b细胞淋巴瘤(DLBCL)。我们报告一例原发性颅拱顶弥漫性大b细胞淋巴瘤(PCV-DLBCL),成功地用蒽环类化学免疫疗法(CIT)与中枢神经系统(CNS)定向CIT交替使用高剂量甲氨蝶呤和高剂量阿糖胞苷治疗。以中枢神经系统为中心的治疗给予怀疑大脑皮层受累和假定中枢神经系统复发的风险升高。在治疗后,患者保持了4.25年的完全缓解。我们认为PCV-DLBCL通过中枢神经系统定向治疗是可以治愈的。此外,我们提供的基因组分析结果表明,不确定的细胞起源和多种基因突变在DLBCL中并不常见。
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来源期刊
CiteScore
3.50
自引率
0.00%
发文量
94
审稿时长
16 weeks
期刊介绍: The Journal of Blood Medicine is an international, peer-reviewed, open access, online journal publishing laboratory, experimental and clinical aspects of all topics pertaining to blood based medicine including but not limited to: Transfusion Medicine (blood components, stem cell transplantation, apheresis, gene based therapeutics), Blood collection, Donor issues, Transmittable diseases, and Blood banking logistics, Immunohematology, Artificial and alternative blood based therapeutics, Hematology including disorders/pathology related to leukocytes/immunology, red cells, platelets and hemostasis, Biotechnology/nanotechnology of blood related medicine, Legal aspects of blood medicine, Historical perspectives. Original research, short reports, reviews, case reports and commentaries are invited.
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