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  • Primer
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Hodgkin lymphoma

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An Author Correction to this article was published on 20 October 2021

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Abstract

Hodgkin lymphoma (HL) is a B cell lymphoma characterized by few malignant cells and numerous immune effector cells in the tumour microenvironment. The incidence of HL is highest in adolescents and young adults, although HL can affect elderly individuals. Diagnosis is based on histological and immunohistochemical analyses of tissue from a lymph node biopsy; the tissue morphology and antigen expression profile enable classification into one of the four types of classic HL (nodular sclerosis, mixed cellularity, lymphocyte-depleted or lymphocyte-rich HL), which account for the majority of cases, or nodular lymphocyte-predominant HL. Although uncommon, HL remains a crucial test case for progress in cancer treatment. HL was among the first systemic neoplasms shown to be curable with radiation therapy and multiagent chemotherapy. The goal of multimodality therapy is to minimize lifelong residual treatment-associated toxicity while maintaining high levels of effectiveness. Recurrent or refractory disease can be effectively treated or cured with high-dose chemotherapy followed by autologous haematopoietic stem cell transplantation, and prospective trials have demonstrated the potency of immunotherapeutic approaches with antibody–drug conjugates and immune checkpoint inhibitors. This Primer explores the wealth of information that has been assembled to understand HL; these updated observations verify that HL investigation and treatment remain at the leading edge of oncological research.

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Fig. 1: Morphological and cellular characteristics of Hodgkin lymphoma.
Fig. 2: Relationship between age and subtype of Hodgkin lymphoma.
Fig. 3: Annual age-specific incidence of Hodgkin lymphoma in different regions.
Fig. 4: Tumour microenvironment.
Fig. 5: Morphological features of HRS cells and EBV infection of HRS cells.
Fig. 6: Management algorithm for classic Hodgkin lymphoma.
Fig. 7: Management algorithm for nodular lymphocyte-predominant Hodgkin lymphoma.
Fig. 8: Freedom from progression in Hodgkin lymphoma.

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Contributions

Introduction (J.M.C.); Epidemiology (W.C., A.C. and J.M.C.); Mechanisms/pathophysiology (C.S., A.C. and J.M.C.); Diagnosis, screening and prevention (A.C., W.C., C.S. and J.M.C.); Management (R.T.H., N.L.B. and J.M.C.); Quality of life (H.-H.F. and J.M.C.); Outlook (N.L.B. and J.M.C.); Overview of Primer (J.M.C.).

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Correspondence to Joseph M. Connors.

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Competing interests

J.M.C. has received research support and honoraria from Seattle Genetics and Takeda Pharmaceuticals; N.L.B. has received research support from Seattle Genetics, Takeda Pharmaceuticals, Bristol-Myers Squibb and Merck, and is on the Advisory Board of ADC Therapeutics and Seattle Genetics. All other authors declare no competing interests.

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Nature Reviews Disease Primers thanks P. Borchmann, L. Castagna, A. Gallamini, R. Jarrett, R. Küppers, A. Sureda and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Connors, J.M., Cozen, W., Steidl, C. et al. Hodgkin lymphoma. Nat Rev Dis Primers 6, 61 (2020). https://doi.org/10.1038/s41572-020-0189-6

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