Piccaluga Pier Paolo, Agostinelli Claudio, Gazzola Anna, Tripodo Claudio, Bacci Francesco, Sabattini Elena, Sista Maria Teresa, Mannu Claudia, Sapienza Maria Rosaria, Rossi Maura, Laginestra Maria Antonella, Sagramoso-Sacchetti Carlo A, Righi Simona, Pileri Stefano A
Hematopathology Section, Department of Hematology and Oncological Sciences "L. and A. Seràgnoli", S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, Italy.
Adv Hematol. 2011;2011:920898. doi: 10.1155/2011/920898. Epub 2010 Dec 22.
Despite its well-known histological and clinical features, Hodgkin's lymphoma (HL) has recently been the object of intense research activity, leading to a better understanding of its phenotype, molecular characteristics, histogenesis, and possible mechanisms of lymphomagenesis. There is complete consensus on the B-cell derivation of the tumor in most cases, and on the relevance of Epstein-Barr virus infection and defective cytokinesis in at least a proportion of patients. The REAL/WHO classification recognizes a basic distinction between lymphocyte predominance HL (LP-HL) and classic HL (cHL), reflecting the differences in clinical presentation and behavior, morphology, phenotype, and molecular features. cHL has been classified into four subtypes: lymphocyte rich, nodular sclerosing, with mixed cellularity, and lymphocyte depleted. The borders between cHL and anaplastic large-cell lymphoma have become sharper, whereas those between LP-HL and T-cell-rich B-cell lymphoma remain ill defined. Treatments adjusted to the pathobiological characteristics of the tumor in at-risk patients have been proposed and are on the way to being applied.
尽管霍奇金淋巴瘤(HL)具有众所周知的组织学和临床特征,但它最近一直是深入研究的对象,这使得人们对其表型、分子特征、组织发生以及淋巴瘤发生的可能机制有了更好的理解。在大多数情况下,对于肿瘤的B细胞起源以及至少一部分患者中爱泼斯坦-巴尔病毒感染和有缺陷的胞质分裂的相关性,已经达成了完全共识。REAL/WHO分类法认识到淋巴细胞为主型HL(LP-HL)和经典HL(cHL)之间的基本区别,这反映了临床表现和行为、形态、表型以及分子特征方面的差异。cHL已被分为四种亚型:富于淋巴细胞型、结节硬化型、混合细胞型和淋巴细胞消减型。cHL与间变性大细胞淋巴瘤之间的界限变得更加清晰,而LP-HL与富于T细胞的B细胞淋巴瘤之间的界限仍然不明确。已经提出了根据高危患者肿瘤的病理生物学特征调整的治疗方法,并且正在应用的过程中。