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1 Pathologic Anatomy and Haematopathology, Bologna University, 40138 Bologna Italy
2 Institute of Pathologic Anatomy and Histopathology, Siena University, 53100 Siena, Italy
3 Institute of Haematology, Perugia University, 06100 Perugia, Italy
4 Institute of Pathologic Anatomy, Perugia University in Terni, 05100 Terni, Italy
5 Institute of Pathology, Berlin Free University, 12200 Berlin, Germany
Correspondence to:
Professor S A Pileri, Anatomia Patologica ed Ematopatologia, Istituto di Ematologia ed Oncologia Medica "L. e A. Seràgnoli", Università di Bologna, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy;
pileri{at}almadns.unibo.it
ABSTRACT
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 tumour 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 recognises a basic distinction between lymphocyte predominance HL (LP-HL) and classic HL (CHL), reflecting the differences in clinical presentation and behaviour, 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 tumour in at risk patients have been proposed and are on the way to being applied.
Keywords: Hodgkin's lymphoma; differential diagnosis; subtypes; Epstein-Barr virus
Abbreviations: ALCL, anaplastic large cell lymphoma; ALCL-HL, ALCL Hodgkin-like type; BNLI, British national lymphoma investigation; CHL, common Hodgkin's lymphoma; DLBCL, diffuse large B cell lymphoma; EBER, Epstein-Barr virus early RNA; EBV, Epstein-Barr virus; EMA, epithelial membrane antigen; FDC, follicular dendritic cell; FL, follicular lymphoma; HC, Hodgkin's cell; HCRBCL, histiocyte rich large B cell lymphoma; HD, Hodgkin's disease; HIV, human immunodeficiency virus; HL, Hodgkin's lymphoma; H&RS, Hodgkin's and Reed-Sternberg; IL, interleukin; LD-CHL, lymphocyte depletion CHL; L/H, lymphocytic/histiocytic; LL, Lennert's lymphoma; LMP, latent membrane protein; LP-HL, lymphocyte predominant HL; LR-CHL, lymphocyte rich CHL; MC-CHL, mixed cellularity CHL; NHL, non-Hodgkin's lymphoma; NS-CHL, nodular sclerosis CHL; PCNA, proliferating cell nuclear antigen; PCR, polymerase chain reaction; PMLBCL, primary mediastinal large B cell lymphoma; PTGC, progressively transformed germinal centre; REAL, revised EuropeanAmerican; RS, Reed-Sternberg; TCR, T cell receptor; TCRBCL, T cell rich B cell lymphoma; TNF, tumour necrosis factor; WHO, World Health Organisation
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