具有致命意义的临床三位一体:复发性弥漫性大b细胞非霍奇金淋巴瘤在白血病期表现为血清乳酸水平升高和肿瘤抑制基因TP53失调-一例报告和文献综述

IF 3 Q2 Medicine Clinical Medicine Insights-Blood Disorders Pub Date : 2021-02-19 eCollection Date: 2021-01-01 DOI:10.1177/2634853521994094
Catherine S Hwang, Dick G Hwang, David M Aboulafia
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引用次数: 3

摘要

尽管弥漫性大b细胞淋巴瘤(DLBCL)占成人非霍奇金淋巴瘤新诊断病例的30%至40%,但它很少出现(1)白血病期(2)TP53肿瘤抑制基因失调和(3)血清乳酸水平升高。在这个病例报告和文献回顾中,我们强调了一个53岁的复发性DLBCL患者的侵袭性和致命的临床过程相关的不良预后特征。新发或复发的DLBCL的白血病表现是罕见的,可能与细胞表面粘附分子的差异表达有关。此外,约20%至25%的DLBCL病例中存在TP53基因突变,预示着较差的临床结果。最后,DLBCL患者血清乳酸水平升高与脓毒症综合征无明显相关性,是预后不良的生存因素,并通过Warburg效应表现为B型乳酸酸中毒。
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A Clinical Triad with Fatal Implications: Recrudescent Diffuse Large B-cell Non-Hodgkin Lymphoma Presenting in the Leukemic Phase with an Elevated Serum Lactic Acid Level and Dysregulation of the TP53 Tumor Suppressor Gene - A Case Report and Literature Review.

Despite representing 30% to 40% of newly diagnosed cases of adult non-Hodgkin lymphoma, diffuse large B-cell lymphoma (DLBCL) rarely presents (1) in the leukemic phase (2) with dysregulation of the TP53 tumor suppressor gene and (3) an elevated serum lactic acid level. In this case report and literature review, we highlight this unfortunate triad of poor prognostic features associated with an aggressive and fatal clinical course in a 53-year-old man with recrudescent DLBCL. A leukemic presentation of de novo or relapsed DLBCL is rare and may be related to differential expressions of adhesion molecules on cell surfaces. In addition, TP53 gene mutations are present in approximately 20% to 25% of DLBCL cases and foreshadow worse clinical outcomes. Finally, an elevated serum lactic acid level in DLBCL that is not clearly associated with sepsis syndrome is a poor prognostic factor for survival and manifests as type B lactic acidosis through the Warburg effect.

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