青少年和成人伯基特淋巴瘤的治疗结果:回顾性研究

Sugeeth Mt, G. Narayanan, Anoop Tm, S. Nair, Prakash Np, J. Km, R. Nair
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摘要

摘要简介:伯基特淋巴瘤(Burkitt’s lymphoma, BL)是一种高度侵袭性的B细胞非霍奇金淋巴瘤(NHL),有三种不同的亚型:地方性、散发性和免疫缺陷相关的BL。散发性BL仅占成人NHL的1%至2%。目的:本文的目的是研究BL患者的临床概况和治疗结果。材料和方法:这是一项回顾性研究,在印度三级癌症中心肿瘤内科进行了10年的60例BL患者。纳入研究期间诊断的14岁以上BL/白血病患者,研究其临床表现、治疗细节和结局。结果:60例BL中,男性41例,女性19例。就诊时的中位年龄为42岁(范围:14-81岁)。主要症状为淋巴结肿大、腹痛、腹胀。两名患者分别有麻痹、乳房肿块和下颌肿胀,一名患者有子宫颈受累。13例患者出现肿瘤溶解特征。安娜堡期为17年1期,16年2期,5年3期,22年4期。55例患者接受联合化疗,包括超环磷酰胺、长春新碱、阿霉素、地塞米松±利妥昔单抗(hyper-CVAD±R;35例)、环磷酰胺、阿霉素、长春新碱、强的松龙/环磷酰胺、长春新碱、强的松龙±利妥昔单抗CHOP±R(13例)、柏林-法兰克福-慕尼黑方案(4例)等(3例)。34例患者获得缓解,13例患者病情进展,8例患者在化疗期间死亡。在中位随访113个月时,58%的患者存活。结论:14岁以上NHL患者中,BL占1.57%,以男性为主。强化、短时间化疗是标准的治疗方法。以hyper-CVAD±R治疗可获得8年无进展生存期,总生存期(OS)为60%。CHOP±R治疗是8年总生存率为46%的老年体弱患者的另一种选择。
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Treatment Outcome of Burkitt's Lymphoma in Adolescents and Adults: A Retrospective Study
Abstract Introduction : Burkitt's lymphoma (BL) is a highly aggressive B cell non-Hodgkin lymphoma (NHL) having three distinct subtypes: endemic, sporadic, and immunodeficiency-associated BL. Sporadic BL accounts for only 1 to 2% of adult NHL. Objectives : The objective of this article was to study the clinical profile and treatment outcome of patients with BL. Materials and Methods : This was a retrospective study of 60 patients with BL conducted in the department of medical oncology at a tertiary cancer center in India during a 10-year period. Patients with BL/leukemia above 14 years of age diagnosed during the study period were included and their clinical presentation, treatment details, and outcome were studied. Results : Among 60 cases with BL, there were 41 males and 19 females. The median age at presentation was 42 years (range: 14–81 years). The main symptoms were lymphadenopathy, abdominal pain, and abdominal distension. Two patients each had paraparesis, breast lump, and jaw swelling and one patient had involvement of the cervix. Thirteen patients had features of tumor lysis at presentation. The Ann Arbor stage was I in 17, II in 16, III in 5, and IV in 22. Fifty-five patients received combination chemotherapy that included hyper-cyclophosphamide, vincristine, adriamycin, dexamethasone ± rituximab (hyper-CVAD ± R; 35), cyclophosphamide, adriamycin, vincristine, prednisolone/ cyclophosphamide, vincristine, prednisolone ± rituximab CHOP ± R (13), Berlin-Frankfurt-Munich protocol (4), and others (3). Thirty-four patients attained remission, 13 patients had progressive disease, and 8 patients died during chemotherapy. At a median follow-up of 113 months, 58% patients were alive. Conclusions : BL accounts for 1.57% of NHL above the age of 14 years with male preponderance. Intensive, short-duration chemotherapy is the standard treatment. Treatment with hyper-CVAD ± R gives 8-year progression-free survival and overall survival (OS) of 60%. Treatment with CHOP ± R is an alternative option in elderly frail patients with an 8-year OS of 46%.
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期刊介绍: The journal will cover technical and clinical studies related to medical and pediatric oncology in human well being including ethical and social issues. Articles with clinical interest and implications will be given preference.
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