Hwang Catherine S, Hwang Dick G, Aboulafia David M
Department of Medicine, Virginia Mason Medical Center, Seattle, WA, USA.
Department of Pathology, Virginia Mason Medical Center, Seattle, WA, USA.
Clin Med Insights Blood Disord. 2021 Feb 19;14:2634853521994094. doi: 10.1177/2634853521994094. eCollection 2021.
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.
尽管弥漫性大B细胞淋巴瘤(DLBCL)占新诊断成人非霍奇金淋巴瘤病例的30%至40%,但很少出现(1)白血病期、(2)TP53肿瘤抑制基因失调以及(3)血清乳酸水平升高的情况。在本病例报告和文献综述中,我们着重介绍了一名53岁复发性DLBCL男性患者中,这种与侵袭性和致命性临床病程相关的不良预后特征的不幸三联征。初发或复发的DLBCL出现白血病表现较为罕见,可能与细胞表面黏附分子的差异表达有关。此外,约20%至25%的DLBCL病例存在TP53基因突变,预示着更差的临床结局。最后,DLBCL中血清乳酸水平升高且与脓毒症综合征无明显关联是生存的不良预后因素,并通过瓦伯格效应表现为B型乳酸酸中毒。