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CD56 阳性弥漫性大 B 细胞淋巴瘤/白血病伴双打击和多个基因突变:预后不良的指标?

CD56-positive diffuse large B-cell lymphoma/leukemia with double-hit and multiple gene mutations: an indicator of poor prognosis?

机构信息

Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, National and Fujian Provincial Key Laboratory of Hematology, Fuzhou, Fujian province, China.

出版信息

J Int Med Res. 2020 May;48(5):300060520918087. doi: 10.1177/0300060520918087.

DOI:10.1177/0300060520918087
PMID:32363958
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7218936/
Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common adult non-Hodgkin lymphoma (NHL) and is highly invasive, with a poor prognosis. The main clinical treatment for DLBCL involves chemotherapy or a combination of chemotherapy and targeted drugs. CD56 expression is considered as an indicator of poor prognosis in patients with acute myeloid leukemia and anaplastic large cell lymphoma; however, its role in DLBCL remains unclear. We report on a patient with CD56-positive DLBCL/leukemia with / double-hit, and , , , and gene mutations (stage IVA, prognostic index aaIPI = 2 points). The patient was treated with cyclophosphamide and prednisone pre-chemotherapy plus R-Hyper-CVAD AB and DA-EPOCH regimens. Lumbar puncture combined with intrathecal injection was performed to prevent central nervous system infiltration during hospitalization, and complete remission was confirmed. We also reviewed the literature to clarify the relevance of the unique clinical features associated with this case.

摘要

弥漫性大 B 细胞淋巴瘤(DLBCL)是最常见的成人非霍奇金淋巴瘤(NHL),具有侵袭性强、预后不良的特点。DLBCL 的主要临床治疗方法包括化疗或化疗联合靶向药物治疗。CD56 表达被认为是急性髓系白血病和间变大细胞淋巴瘤患者预后不良的指标;然而,其在 DLBCL 中的作用尚不清楚。我们报告了一例 CD56 阳性 DLBCL/白血病伴双打击,和 、 、 、 和 基因突变(IV 期 A,预后指数 aaIPI = 2 分)的患者。该患者接受了环磷酰胺和泼尼松化疗前治疗,加用 R-Hyper-CVAD AB 和 DA-EPOCH 方案。住院期间行腰椎穿刺联合鞘内注射,以预防中枢神经系统浸润,并确认完全缓解。我们还回顾了文献,以阐明与该病例相关的独特临床特征的相关性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/c0d8fc949f1e/10.1177_0300060520918087-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/2c8868ce7602/10.1177_0300060520918087-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/da6b23ae0bbf/10.1177_0300060520918087-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/c0d8fc949f1e/10.1177_0300060520918087-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/2c8868ce7602/10.1177_0300060520918087-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/da6b23ae0bbf/10.1177_0300060520918087-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59b4/7218936/c0d8fc949f1e/10.1177_0300060520918087-fig3.jpg

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