Outlook
Using 2014-2020 data, the National Cancer Institute Surveillance, Epidemiology, and End Results Program reports a 5-year relative survival rate of 88.9% among patients with any stage of HL at diagnosis.[153]National Cancer Institute; Surveillance, Epidemiology, and End Results Program. Hodgkin lymphoma: SEER 5-year relative survival rates, 2012-2018 [internet publication].
https://seer.cancer.gov/statistics-network/explorer/application.html?site=83&data_type=4&graph_type=5&compareBy=sex&chk_sex_1=1&series=9&race=1&age_range=1&stage=101&advopt_precision=1&advopt_show_ci=on&hdn_view=1&advopt_show_apc=on&advopt_display=2#tableWrap
Early HL (stage I to stage II)
The prognosis for patients with early-stage HL is excellent with long-term disease control of 80% to 90% following combined-modality therapy (i.e., combination chemotherapy followed by low-dose involved-field radiation therapy [IFRT]).[51]Meyer RM, Gospodarowicz MK, Connors JM, et al. Randomized comparison of ABVD chemotherapy with a strategy that includes radiation therapy in patients with limited-stage Hodgkin's lymphoma: National Cancer Institute of Canada Clinical Trials Group and the Eastern Cooperative Oncology Group. J Clin Oncol. 2005 Jul 20;23(21):4634-42.
https://ascopubs.org/doi/full/10.1200/jco.2005.09.085
http://www.ncbi.nlm.nih.gov/pubmed/15837968?tool=bestpractice.com
[55]Noordijk EM, Carde P, Dupouy N, et al. Combined-modality therapy for clinical stage I or II Hodgkin's lymphoma: long-term results of the European Organisation for Research and Treatment of Cancer H7 randomized controlled trials. J Clin Oncol. 2006 Jul 1;24(19):3128-35.
https://ascopubs.org/doi/full/10.1200/jco.2005.05.2746
http://www.ncbi.nlm.nih.gov/pubmed/16754934?tool=bestpractice.com
[154]Engert A, Schiller P, Josting A, et al. Involved-field radiotherapy is equally effective and less toxic compared with extended-field radiotherapy after four cycles of chemotherapy in patients with early-stage unfavorable Hodgkin's lymphoma: results of the HD8 trial of the German Hodgkin's Lymphoma Study Group. J Clin Oncol. 2003 Oct 1;21(19):3601-8.
http://www.ncbi.nlm.nih.gov/pubmed/12913100?tool=bestpractice.com
While recurrent HL is the leading cause of death for the first 15 years after treatment, with continued follow-up patients are more likely to die of secondary malignancies or cardiac disease.[155]Ng AK, Bernardo MP, Weller E, et al. Long-term survival and competing causes of death in patients with early-stage Hodgkin's disease treated at age 50 or younger. J Clin Oncol. 2002 Apr 15;20(8):2101-8.
http://www.ncbi.nlm.nih.gov/pubmed/11956271?tool=bestpractice.com
Newer treatment approaches aim to reduce the intensity of treatment yet maintain high cure rates. These include the use of involved-site radiation therapy (ISRT) instead of IFRT. ISRT focuses radiation only on involved lymph nodes and nearby sites, minimizing radiation exposure to uninvolved structures and reducing the risk of adverse effects (e.g., secondary malignancies, cardiovascular disease, decreased pulmonary function). Although the evidence for ISRT in HL is evolving, it is the preferred approach and current standard of care.[33]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: Hodgkin lymphoma [internet publication].
https://www.nccn.org/professionals/physician_gls/default.aspx
[70]Specht L, Yahalom J, Illidge T, et al. Modern radiation therapy for Hodgkin lymphoma: field and dose guidelines from the international lymphoma radiation oncology group (ILROG). Int J Radiat Oncol Biol Phys. 2014 Jul 15;89(4):854-62.
http://www.ncbi.nlm.nih.gov/pubmed/23790512?tool=bestpractice.com
[71]Roberts KB, Younes A, Hodgson DC, et al. ACR appropriateness criteria® Hodgkin lymphoma-unfavorable clinical stage I and II. Am J Clin Oncol. 2016 Aug;39(4):384-95.
https://www.doi.org/10.1097/COC.0000000000000294
http://www.ncbi.nlm.nih.gov/pubmed/27299425?tool=bestpractice.com
[72]Kamran SC, Jacene HA, Chen YH, et al. Clinical outcome of patients with early stage favorable Hodgkin lymphoma treated with ABVD × two cycles followed by FDG-PET/CT restaging and 20 Gy of involved-site radiotherapy. Leuk Lymphoma. 2018 Jun;59(6):1384-90.
http://www.ncbi.nlm.nih.gov/pubmed/28937297?tool=bestpractice.com
[73]Wirth A, Mikhaeel NG, Aleman BMP, et al. Involved site radiation therapy in adult lymphomas: an overview of International Lymphoma Radiation Oncology Group guidelines. Int J Radiat Oncol Biol Phys. 2020 Aug 1;107(5):909-33.
https://www.doi.org/10.1016/j.ijrobp.2020.03.019
http://www.ncbi.nlm.nih.gov/pubmed/32272184?tool=bestpractice.com
Advanced HL (stage III to stage IV)
Advanced HL is a heterogeneous disease. Overall, the long-term disease control after chemotherapy alone or combined-modality therapy is approximately 60% to 80%.[156]Diehl V, Franklin J, Pfreundschuh M, et al. Standard and increased-dose BEACOPP chemotherapy compared with COPP-ABVD for advanced Hodgkin's disease. N Engl J Med. 2003 Jun 12;348(24):2386-95. [Erratum in: N Engl J Med. 2005 Aug 18;353(7):744.]
https://www.nejm.org/doi/full/10.1056/NEJMoa022473
http://www.ncbi.nlm.nih.gov/pubmed/12802024?tool=bestpractice.com
[157]Canellos GP, Anderson JR, Propert KJ, et al. Chemotherapy of advanced Hodgkin's disease with MOPP, ABVD, or MOPP alternating with ABVD. N Engl J Med. 1992 Nov 19;327(21):1478-84.
http://www.ncbi.nlm.nih.gov/pubmed/1383821?tool=bestpractice.com
[158]Gobbi PG, Levis A, Chisesi T, et al. ABVD versus modified Stanford V versus MOPPEBVCAD with optional and limited radiotherapy in intermediate- and advanced-stage Hodgkin's lymphoma: final results of a multicenter randomized trial by the Intergruppo Italiano Linfomi. J Clin Oncol. 2005 Dec 20;23(36):9198-207.
https://ascopubs.org/doi/full/10.1200/jco.2005.02.907
http://www.ncbi.nlm.nih.gov/pubmed/16172458?tool=bestpractice.com
Nodular lymphocyte-predominant HL (NLPHL)
Most patients with NLPHL present with asymptomatic early (stage I to II) disease. Overall prognosis for patients with NLPHL is good, particularly for early-stage disease. Long-term disease control with current treatment strategies is approximately 80% to 90% for early-stage disease.[132]Eichenauer DA, Plütschow A, Fuchs M, et al. Long-term course of patients with stage IA nodular lymphocyte-predominant Hodgkin lymphoma: a report from the German Hodgkin study group. J Clin Oncol. 2015 Sep 10;33(26):2857-62.
https://www.doi.org/10.1200/JCO.2014.60.4363
http://www.ncbi.nlm.nih.gov/pubmed/26240235?tool=bestpractice.com
[159]Schlembach PJ, Wilder RB, Jones D, et al. Radiotherapy alone for lymphocyte-predominant Hodgkin's disease. Cancer J. 2002 Sep-Oct;8(5):377-83.
http://www.ncbi.nlm.nih.gov/pubmed/12416895?tool=bestpractice.com
[160]Wirth A, Yuen K, Barton M, et al. Long-term outcome after radiotherapy alone for lymphocyte-predominant Hodgkin lymphoma: a retrospective multicenter study of the Australasian Radiation Oncology Lymphoma Group. Cancer. 2005 Sep 15;104(6):1221-9.
https://www.doi.org/10.1002/cncr.21303
http://www.ncbi.nlm.nih.gov/pubmed/16094666?tool=bestpractice.com
[161]Chen RC, Chin MS, Ng AK, et al. Early-stage, lymphocyte-predominant Hodgkin's lymphoma: patient outcomes from a large, single-institution series with long follow-up. J Clin Oncol. 2010 Jan 1;28(1):136-41.
https://www.doi.org/10.1200/JCO.2009.24.0945
http://www.ncbi.nlm.nih.gov/pubmed/19933914?tool=bestpractice.com