Classic Hodgkin lymphoma in young people

被引:3
作者
Gupta, Srishti [1 ]
Craig, Jeffrey W. [1 ]
机构
[1] Univ Virginia Hlth Syst, Dept Pathol, 1215 Lee St,3rd Floor Hosp Expans Room 3032,POB 80, Charlottesville, VA 22908 USA
关键词
Hodgkin lymphoma; Risk factor; Epstein Barr virus; Mutational signature; Pediatric; Pathogenesis; EPSTEIN-BARR-VIRUS; REED-STERNBERG CELLS; GENOME-WIDE ASSOCIATION; CENTER B-CELL; HLA-CLASS-I; IMMUNOGLOBULIN GENE REARRANGEMENTS; CHILDHOOD SOCIAL-ENVIRONMENT; TUMOR-ASSOCIATED MACROPHAGES; LATENT MEMBRANE PROTEIN-1; ABERRANT EXPRESSION;
D O I
10.1053/j.semdp.2023.06.005
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
Classic Hodgkin lymphoma (CHL) is a unique form of lymphoid cancer featuring a heterogeneous tumor microenvironment and a relative paucity of malignant Hodgkin and Reed-Sternberg (HRS) cells with characteristic phenotype. Younger individuals (children, adolescents and young adults) are affected as often as the elderly, producing a peculiar bimodal age-incidence profile that has generated immense interest in this disease and its origins. Decades of epidemiological investigations have documented the populations most susceptible and identified multiple risk factors that can be broadly categorized as either biological or environmental in nature. Most risk factors result in overt immunodeficiency or confer more subtle alterations to baseline health, physiology or immune function. Epstein Barr virus, however, is both a risk factor and well-established driver of lymphomagenesis in a significant subset of cases. Epigenetic changes, along with the accumulation of somatic driver mutations and cytogenetic abnormalities are required for the malignant transformation of germinal center-experienced HRS cell precursors. Chromosomal instability and the influence of endogenous mutational processes are critical in this regard, by impacting genes involved in key signaling pathways that promote the survival and proliferation of HRS cells and their escape from immune destruction. Here we review the principal features, known risk factors and lymphomagenic mechanisms relevant to newly diagnosed CHL, with an emphasis on those most applicable to young people.
引用
收藏
页码:379 / 391
页数:13
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