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成人伯基特淋巴瘤——淋巴瘤与白血病之间的一个“孤岛”

Adult Burkitt lymphoma- an Island between lymphomas and leukemias.

作者信息

Turro James, Singh Pratiksha, Sarao Manbeer Singh, Tadepalli Satish, Cheriyath Pramil

机构信息

Department of Internal Medicine, Hackensack Meridian Health- Ocean Medical Centre, Brick, NJ, USA.

出版信息

J Community Hosp Intern Med Perspect. 2019 Feb 11;9(1):25-28. doi: 10.1080/20009666.2019.1574545. eCollection 2019.

Abstract

: Burkitt lymphoma is a rare, aggressive and rapidly fatal, B-cell non-Hodgkin's lymphoma. It has an incidence of 0.4/100,000 age-adjusted to the USA standard population. Here we describe the case of a 77-year-old patient who presented with Burkitt lymphoma. : A 77-year-old male presented to his primary care physician with fatigue and listlessness and was referred to the hospital with a white blood cell count (WBC)-23.7 K/uL (neutrophils 37%, lymphocyte 11%, blasts 9%) and platelets-19 K/uL. During his stay in the hospital, repeat investigations revealed WBC-29.9 K/uL (neutrophils 22%, lymphocyte 27%, atypical lymphocytes 5%, blasts 20%) and platelets-10 K/uL with no evidence of mucosal bleeds, neck or abdominal masses or generalized lymphadenopathy. Bone marrow aspirate revealed the presence of MYC rearrangements (8q24) on flow cytometry and fluorescent in-situ hybridization (FISH), indicative but not typical of BL. He was transfused with platelets due to a rapidly deteriorating platelet count and episodes of epistaxis. He was discharged after four days with a plan of outpatient chemotherapy over a period of 4 months. An Ommaya reservoir was placed in the right ventricle for intrathecal chemotherapy. After four months of chemotherapy, computerized tomography of the chest, abdomen, and pelvis confirmed remission. A magnetic resonance imaging of the brain a month after completion of chemotherapy revealed metastatic lymphoma in the temporal, parietal and occipital lobes. He was discharged to hospice for palliative care. : Unconventional presentations, as seen in our case of a leukemia-like picture in the absence of a bulky disease, are the quagmire that might delay aggressive management and result in poorer outcomes.

摘要

伯基特淋巴瘤是一种罕见、侵袭性强且迅速致命的B细胞非霍奇金淋巴瘤。根据美国标准人群年龄调整后,其发病率为0.4/10万。在此,我们描述一例77岁伯基特淋巴瘤患者的病例。一名77岁男性因疲劳和倦怠就诊于其初级保健医生,随后因白细胞计数(WBC)为23.7K/μL(中性粒细胞37%,淋巴细胞11%,原始细胞9%)和血小板计数为19K/μL被转诊至医院。在住院期间,复查结果显示白细胞计数为29.9K/μL(中性粒细胞22%,淋巴细胞27%,非典型淋巴细胞5%,原始细胞20%),血小板计数为10K/μL,且无黏膜出血、颈部或腹部肿块或全身淋巴结肿大的迹象。骨髓穿刺显示在流式细胞术和荧光原位杂交(FISH)检测中存在MYC重排(8q24),提示为伯基特淋巴瘤但不典型。由于血小板计数迅速下降和鼻出血发作,他接受了血小板输注。四天后出院,计划在4个月内进行门诊化疗。在右心室置入了一个Ommaya储液器用于鞘内化疗。化疗四个月后,胸部、腹部和骨盆的计算机断层扫描证实病情缓解。化疗完成一个月后,脑部磁共振成像显示颞叶、顶叶和枕叶有转移性淋巴瘤。他被转至临终关怀机构接受姑息治疗。非典型表现,如我们病例中在无大块病变情况下出现白血病样表现,是可能延迟积极治疗并导致较差预后的困境。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bb62/6374956/14c1521d7a3e/ZJCH_A_1574545_F0001_OC.jpg

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Burkitt lymphoma in adolescents and young adults: management challenges.青少年和青年的伯基特淋巴瘤:管理挑战
Adolesc Health Med Ther. 2016 Dec 23;8:11-29. doi: 10.2147/AHMT.S94170. eCollection 2017.
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