sporadic burkitt lymphoma

散发性伯基特淋巴瘤
  • 文章类型: Case Reports
    成人肠套叠很少发生,通常是由良性或恶性病因的可识别的导联引起的。这里,我们介绍了一名19岁的男性,他向急诊科就诊,抱怨腹痛,顽固性恶心,以及血性腹泻和便秘之间的波动。这些症状在两个月前就开始了,并且严重程度有所增加,导致明显的食欲变化。获得了腹部和盆腔计算机断层扫描,没有造影,其中显示了回盲肠肠套叠进入横结肠并导致小肠梗阻的证据。病人做了剖腹探查术,由于存在6.8厘米的盲肠肿块并可触及肠系膜淋巴结肿大,导致部分回肠结肠切除术。根据组织学组合,病理标本被鉴定为伯基特淋巴瘤,免疫组织化学,和荧光原位杂交结果。目前,病人正在接受三个周期的利妥昔单抗,环磷酰胺,长春新碱,阿霉素,大剂量甲氨蝶呤,异环磷酰胺,依托泊苷,和大剂量阿糖胞苷(R-CODOX-M/IVAC)按照Magrath方案治疗低危Burkitt淋巴瘤。
    Adult intussusception is an infrequent occurrence typically resulting from an identifiable lead point of a benign or malignant etiology. Here, we present a case of a 19-year-old male who presented to the emergency department with complaints of abdominal pain, intractable nausea, and fluctuations between bloody diarrhea and constipation. These symptoms had begun two months prior and had increased in severity, resulting in significant appetite changes. An abdominal and pelvic computed tomography scan without contrast was obtained, which showed evidence of intussusception of the ileocecum into the transverse colon with resultant small bowel obstruction. The patient underwent an exploratory laparotomy, which resulted in a partial ileocolectomy due to the presence of a 6.8 cm cecal mass with palpable mesenteric lymphadenopathy. The pathologic specimen was identified as Burkitt lymphoma based on a combination of histologic, immunohistochemical, and fluorescence in situ hybridization findings. Currently, the patient is undergoing three cycles of rituximab, cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate, ifosfamide, etoposide, and high-dose cytarabine (R-CODOX-M/IVAC) per Magrath protocol for low-risk Burkitt lymphoma.
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  • 文章类型: Case Reports
    伯基特淋巴瘤(BL)是淋巴组织的肿瘤,是全球最常见的恶性肿瘤之一。经典的,这些患者表现为B细胞分化失调导致发热,发冷,盗汗,和减肥。虽然在儿童中更常见,散发性伯基特淋巴瘤,症状常出现在腹部。这些患者还报告恶心,呕吐,和腹胀,在极少数情况下会导致小肠梗阻(SBO)。早期检测和开始化疗在提供充分护理方面仍然非常有效。这提供了更好的结果并阻止了手术管理。
    Burkitt lymphoma (BL) is a neoplasm of the lymphoid tissue and one of the most prevalent malignancies worldwide. Classically, these patients present with unregulated B-cell differentiation causing fever, chills, night sweats, and weight loss. Although more common in children, in sporadic Burkitt lymphoma, symptoms often can be present in the abdomen. These patients also additionally report nausea, vomiting, and abdominal distention, which in rare instances can cause small bowel obstruction (SBO). Early detection and the initiation of chemotherapy remain highly effective in providing adequate care. This provides better outcomes and prevents surgical management.
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  • 文章类型: Case Reports
    伯基特淋巴瘤(BL)的表现是可变的,并且高度依赖于其亚型。它通常表现为非特异性症状,可能出现在下巴,骨髓,肝脏,脾,脾肾脏,卵巢,和胃肠道。该病例描述了一名50岁的男性,他表现出腹痛,最终被发现患有伯基特淋巴瘤,通过盲肠息肉活检诊断。在这里,我们探讨了BL的诊断历程,并回顾了有关其独特表现以及可能带来的诊断挑战的文献.
    The presentation of Burkitt lymphoma (BL) is variable and highly dependent on its subtype. It often presents with non-specific symptoms and may appear in the jaw, bone marrow, liver, spleen, kidneys, ovaries, and gastrointestinal tract. This case describes a 50-year-old male who presented with abdominal pain and was eventually found to have Burkitt lymphoma, diagnosed by biopsy of a cecal polyp. Herein, we explore the diagnostic journey to BL and review the literature regarding its unique manifestations and thus the diagnostic challenge it can present.
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  • 文章类型: Case Reports
    非霍奇金淋巴瘤由B细胞谱系组成,包括结节外边缘淋巴瘤,滤泡性淋巴瘤,套细胞淋巴瘤,弥漫性大B细胞淋巴瘤,和伯基特淋巴瘤.伯基特淋巴瘤与EB病毒和人类免疫缺陷病毒有关。尽管其他B细胞淋巴瘤在胃中发展很常见,伯基特淋巴瘤在那里表现不太常见。此外,十二指肠的原发性和/或继发性受累,胰腺,肠在伯基特淋巴瘤中非常罕见。在这里,我们介绍了一名男性被诊断患有广泛的骨Burkitt淋巴瘤,淋巴结,胰腺,小肠,十二指肠,和胃。
    Non-Hodgkin lymphoma is made from the B-cell lineage and includes extra-nodal marginal lymphomas, follicular lymphomas, mantle cell lymphoma, diffuse large B-cell lymphoma, and Burkitt lymphoma. Burkitt lymphoma is associated with Epstein Barr Virus and Human Immunodeficiency Virus. Although it is common for other B-cell lymphomas to develop in the stomach, it is less common for Burkitt lymphoma tumors to manifest there. Additionally, primary and/or secondary involvement of the duodenum, pancreas, and intestines is very rare in Burkitt lymphoma. Herein, we present a male diagnosed with extensive Burkitt lymphoma of the bone, lymph nodes, pancreas, small intestine, duodenum, and stomach.
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  • 文章类型: Journal Article
    研究以R-CODOX-M/IVAC为基础的改良化疗方案以降低中国成年散发性Burkitt淋巴瘤患者的严重不良事件。
    我们纳入了2008年8月至2019年9月在中山大学肿瘤中心接受治疗的123例未经治疗的散发性Burkitt淋巴瘤成年患者的回顾性队列。我们研究了使用1.0g/m2/周期的低剂量环磷酰胺的剂量修改和长期的R-CODOX-M/IVAC方案,2g/m2/周期甲氨蝶呤,4,500毫克/平方米/周期异环磷酰胺,和4.0g/m2/周期阿糖胞苷。49例低危疾病患者接受了4-6个周期的基于剂量修改的R-CODOX-M化疗。74例高危疾病患者接受了6-8个周期的剂量修改的交替R-CODOX-M/IVAC方案。
    客观缓解率为87.0%。3年无事件生存率和总生存率分别为81.2%和92.1%,分别。主要3-4级不良事件包括白细胞减少症(91.9%),贫血(58.5%),血小板减少症(73.2%),和发热性中性粒细胞减少(48.8%)。分别有26.0%和37.4%的患者接受了红细胞和血小板输注,分别。我们观察到4例(3.3%)化疗后发生感染性休克。2例治疗相关死亡发生于严重感染。
    改良R-CODOX-M/IVAC化疗方案对中国人群散发性伯基特淋巴瘤有效,毒性低于标准方案。
    To characterize modified R-CODOX-M/IVAC-based chemotherapy to lower the severe adverse events in Chinese adult patients with sporadic Burkitt lymphoma.
    We enrolled a retrospective cohort including 123 adult patients with untreated sporadic Burkitt lymphoma from August 2008 to September 2019 at Sun Yat-sen University Cancer Center. We studied a dose-modified and long-course R-CODOX-M/IVAC regimen utilizing a low dose of 1.0 g/m2/cycle cyclophosphamide, 2 g/m2/cycle methotrexate, 4,500 mg/m2/cycle ifosfamide, and 4.0 g/m2/cycle cytarabine. Forty-nine patients with low risk disease underwent 4-6 cycles of dose-modified R-CODOX-M-based chemotherapy. Seventy-four patients with high risk disease underwent 6-8 cycles of dose-modified alternating R-CODOX-M/IVAC regimens.
    The objective remission was 87.0%. The event-free survival rate and overall survival at 3 years were 81.2% and 92.1%, respectively. Major grade 3-4 adverse events included leukopenia (91.9%), anemia (58.5%), thrombocytopenia (73.2%), and febrile neutropenia (48.8%). A total of 26.0% and 37.4% of patients received red blood cell and platelet transfusions, respectively. We observed 4 cases (3.3%) of septic shock after chemotherapy. Two treatment-related deaths occurred from severe infection.
    The modified R-CODOX-M/IVAC chemotherapy regimen was effective for sporadic Burkitt lymphoma in the Chinese population, with a lower toxicity than standard regimens.
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  • 文章类型: Case Reports
    伯基特淋巴瘤(BL)是一种侵袭性淋巴瘤,由于c-myc原癌基因在8号染色体上的易位而发生。BL的特点是三个不同的群体:非洲/地方性变异,免疫抑制变体,或零星变体。大多数散发性变异病例发生在40岁以下的患者中,诊断时的中位年龄为30岁,主要见于白种人。一名具有免疫能力的69岁男性,下肢出现亚急性发作无力。腰椎的磁共振成像(MRI)显示右侧椎旁肌系统有硬膜外伸,神经椎间孔狭窄,L2-L5严重椎管狭窄。胸椎的MRI显示,由于转移性肿块,T5-T6脊髓受到明显压迫。进一步的诊断成像显示纵隔和腹部弥漫性淋巴结肿大。随后,患者接受了左腋窝淋巴结的芯针活检,显示分化簇20和10(CD20和CD10),c-myc,B细胞淋巴瘤6(Bcl-6)阳性淋巴样细胞。诊断为BL。患者接受口服类固醇治疗,并接受了一轮放射治疗。患者选择放弃任何抗肿瘤治疗,并出院到临终关怀医院。中枢神经系统(CNS)的原发性淋巴瘤占所有CNS肿瘤的<5%。大约5-10%的中枢神经系统淋巴瘤记录为BL,大多数被归类为高级别B细胞淋巴瘤。BL的椎旁受累很少见,在散发性变体中并不常见。
    Burkitt lymphoma (BL) is an aggressive form of lymphoma that occurs due to translocation of the c-myc proto-oncogene on chromosome 8. BL is characterized by three distinct groups: African/endemic variant, immunosuppressive variant, or sporadic variant. Most cases of the sporadic variant occur in patients less than 40 years of age with a median age of 30 at diagnosis and are primarily seen in Caucasians. An immunocompetent 69-year-old male presented with subacute onset weakness in the lower extremities. Magnetic resonance imaging (MRI) of the lumbar spine revealed a mass in the right paraspinal musculature with epidural extension, neural foraminal narrowing, and severe spinal canal stenosis in L2-L5. MRI of the thoracic spine revealed significant T5-T6 cord compression due to metastatic masses. Further diagnostic imaging revealed diffuse lymphadenopathy within the mediastinum and abdomen. Subsequently, the patient underwent a core needle biopsy of the left axillary lymph node, which revealed cluster of differentiation 20 and 10 (CD20 and CD10), c-myc, and B-cell lymphoma 6 (Bcl-6) positive lymphoid cells. A diagnosis of BL was made. The patient was treated with oral steroids and received one round of radiation therapy. The patient opted to forgo any antitumor treatment and was discharged to hospice. Primary lymphomas of the central nervous system (CNS) account for <5% of all CNS tumors. Approximately 5-10% of CNS lymphomas are recorded as BL, with the majority classified as high-grade B-cell lymphomas. Paraspinal involvement with BL is rare and not commonly seen in the sporadic variant.
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  • 文章类型: Journal Article
    BACKGROUND: Adult sporadic Burkitt lymphoma (BL) is a rare but highly aggressive subtype of lymphoma which lacks its own unique prognostic model. Systemic inflammatory biomarkers have been confirmed as prognostic markers in several types of malignancy. Our objective was to explore the predictive value of pretreatment inflammatory biomarkers and establish a novel, clinically applicable prognostic index for adult patients with sporadic BL.
    METHODS: We surveyed retrospectively 336 adult patients with newly diagnosed sporadic BL at 8 Chinese medical centers and divided into training cohort (n = 229) and validation cohort (n = 107). The pretreatment inflammatory biomarkers were calculated for optimal cut-off value. The association between serum biomarkers and overall survival (OS) was analyzed by Kaplan-Meier curves and Cox proportional models. The risk stratification was defined based on normal LDH level, Ann Arbor stage of I and completely resected abdominal lesion or single extra-abdominal mass < 10 cm.
    CONCLUSIONS: Univariate and multivariate analyses revealed that platelets< 254 × 109/L, albumin< 40 g/L, lactate dehydrogenase≥334 U/L independently predicted unfavorable OS. We used these data as the basis for the prognostic index, in which patients were stratified into Group 1 (no or one risk factor), Group 2 (two risk factors), or Group 3 (three risk factors), which were associated with 5-year OS rates of 88.1, 72.4, and 45%, respectively. In the subgroup analysis for high-risk patients, our prognostic model results showed that high-risk patients with no more than one adverse factor presented a 5-year survival rate of 85.9%, but patients with three adverse factors had a 5-year survival rate of 43.0%. Harrell\'s concordance index (C-index) of the risk group score was 0.768. Therefore, the new prognostic model could be used to develop risk-adapted treatment approaches for adult sporadic BL.
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  • 文章类型: Case Reports
    BACKGROUND: Acute small bowel obstruction is a common surgical emergency usually caused by abdominal adhesions, followed by intraluminal tumors from metastatic disease. Although lymphomas have been known to cause bowel obstruction, Burkitt lymphoma is seldom reported to induce an obstruction in the adult population.
    METHODS: A 78-year-old Hispanic man with a history of abdominal interventions presented to our hospital with abdominal pain. Computed tomography revealed a partial small bowel obstruction attributed to local inflammation or adhesions. Medical management with bowel rest and nasogastric decompression resulted in resolution of symptoms and quick discharge. He returned 2 days later with worsening abdominal pain. Repeat imaging showed progression of the partial small bowel obstruction, but with an additional 1.6-cm nodular density abutting the anterior aspect of the gastric antrum and lobulated anterior gastric antral wall thickening. He was taken to the operating room, where several masses were found. Intraoperative frozen sections were consistent with lymphoma, and pathology later revealed Burkitt lymphoma. Disease was found on both sides of the diaphragm by positron emission tomography. After the initial resection and adjuvant chemotherapy, the patient is alive and well about 14 months after resection.
    CONCLUSIONS: Small bowel obstruction is uncommonly due to Burkitt lymphoma in the geriatric population and is more frequently seen in the pediatric and young adult populations. Burkitt lymphoma is very aggressive with rapid cell turnover leading to significant morbidity. The rapid recurrence of an acute abdominal process should prompt an investigation for a more sinister cause such as malignancy.
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  • 文章类型: Journal Article
    OBJECTIVE: Sporadic Burkitt lymphoma is rarely associated with orbital involvement.
    METHODS: We present a case of sporadic Burkitt lymphoma with extramedullary subperiosteal leukemic infiltrates of the orbit and facial bones.
    RESULTS: Follow-up examination after chemotherapy and disease remission reveals resolution of the subperiosteal infiltrates.
    CONCLUSIONS: Despite an aggressive presentation, cure is common with appropriate, intensive treatment. To our knowledge, this report is the first to photographically depict the resolution of extramedullary orbital subperiosteal leukemic infiltrates after appropriate chemotherapy.
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