Guideline sources The following summarized guidelines for the evaluation and management of Hodgkin's lymphoma (HL) are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2014). 2 5 6 7 8 8 Definition HL is a rare lymphatic neoplasm, typically of B-cell origin, characterized by large malignant lymphoid cells, and Reed-Sternberg cells. 5 Epidemiology HL is most frequently caused by the EBV. Other causes include tuberculosis and genetic factors (HLA class II polymorphisms). 7 Pathophysiology HL incidence in the United States is estimated at 3.23 per 100,000 people per year. 6 Disease course https://web.pathway.md/diseases/recbCbpohsw9AgeZT 1/5 6/24/23, 12:45 PM Hodgkin's lymphoma Pathway Clinical manifestations include painless lymphadenopathy enlarging over months, mainly involving the mediastinum, left neck node, right neck node, splenic, axillary, abdominal, hilar, or inguinofemoral nodes in descending order. The clinically occult disease is characterized by severe unremitting pruritus without obvious skin pathology. B symptoms include fever, night sweats, and unexplained weight loss > 10% of body weight. Alcohol-induced lymph node pain is highly specific for HL. 8 Prognosis and risk of recurrence Prognosis is determined by disease stage (i.e. best prognosis in early-stage HL) and pathology (i.e. better prognosis in Nodular sclerosis cHL). 8 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Guidelines 1. Classification and risk stratification Severity classification: Classify patients with early-stage HL as having favorable or unfavorable disease. A Classify patients with advanced stage HL using the Hasenclever/International Prognostic Score. A 2. Diagnostic investigations Pretreatment blood tests: obtain pre-treatment blood evaluation, which should include human immunodeficiency virus serology. A Computed tomography of the neck to pelvis: obtain staging with contrast-enhanced CT of the neck to pelvis. A Pretreatment positron emission tomography: obtain positron emission tomography/CT for pre- treatment staging when clinically feasible. B Interim positron emission tomography: obtain interim positron emission tomography as it is highly predictive of outcome in patients treated with adriamycin, bleomycin sulfate, vinblastine sulfate, and dacarbazine. A 3. Diagnostic procedures https://web.pathway.md/diseases/recbCbpohsw9AgeZT 2/5 6/24/23, 12:45 PM Hodgkin's lymphoma Pathway Tissue biopsy: perform biopsy assessment or close clinical/radiological surveillance for early progression in patients who remain positron emission tomography-positive on completion of therapy. B 4. Medical management ABVD regimen: administer doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and radiotherapy (20 Gy) as standard of care in patients with favorable early stage HL. A Concurrent chemoradiation: initiate 4 3 adriamycin, bleomycin sulfate, vinblastine sulfate, and dacarbazine and 30 Gy radiotherapy as the standard of care for unfavorable early stage HL. A Radiotherapy: avoid omitting radiotherapy in patients presenting with bulky disease. D BEACOPP regimen: administer either 6-8 cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine or six cycles of escalated bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP) to patients 16-60 years of age with advanced stage HL. A Irradiated blood products: administer irradiated blood products for life. B 5. Nonpharmacologic interventions Lifestyle modification: offer regular lifestyle advice to reduce secondary neoplasms and cardiovascular risk. Advise complete avoidance of smoking and carefully manage cardiovascular risk factors, such as hypertension, diabetes mellitus and dyslipidemia. B 6. Therapeutic procedures Pretreatment semen cryopreservation: offer pre-treatment semen cryopreservation, if possible, to male patients with HL. A 7. Specific circumstances Pregnant patients: co-manage patients closely with a specialized obstetric/fetal medicine unit. B Show 4 more Elderly patients: assess elderly patients formally to receive combination chemotherapy with a comorbidity assessment tool, to distinguish frail' from non-frail' patients. B 8. Follow-up and surveillance Indications for specialist referral: consider pre-treatment consultation with a fertility specialist for female patients with HL. B https://web.pathway.md/diseases/recbCbpohsw9AgeZT 3/5 6/24/23, 12:45 PM Hodgkin's lymphoma Pathway Serial clinical assessment: follow patients with intermittent outpatient clinical review for 2-5 years following first-line therapy. B Serial imaging assessment: routine surveillance CT or positron emission tomography/CT imaging have no proven role in patients who are otherwise well following first line therapy. B Screening for secondary malignancies: educate hodgkin's lymphoma patients that they are at an increased lifetime risk of second neoplasms, cardiovascular and pulmonary disease and infertility. A Serial laboratory assessment: monitor thyroid function regularly in patients who have had radiotherapy to the neck and upper mediastinum. A References 1. Eichenauer DA, Aleman BMP, Andr M et al. Hodgkin lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 Suppl 4):iv19-iv29. Open 2. Follows GA, Ardeshna KM, Barrington SF et al. Guidelines for the first line management of classical Hodgkin lymphoma. Br J Haematol. 2014 Jul;166 1 34 49. Open 3. Hoppe RT, Advani RH, Ai WZ et al. NCCN Guidelines Insights: Hodgkin Lymphoma, Version 1.2018. J Natl Compr Canc Netw. 2018 Mar;16 3 245 254. Open 4. Eichenauer DA, Engert A, Andr M et al. Hodgkin's lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep;25 Suppl 3:iii70 5. Open 5. Stephen M Ansell. Hodgkin Lymphoma: Diagnosis and Treatment. 2015 Nov;90 11 1574 83.2015 Nov;90 11 1574 83. Open 6. Linghui Zhou, Yujiao Deng, Na Li et al. Global, regional, and national burden of Hodgkin lymphoma from 1990 to 2017 estimates from the 2017 Global Burden of Disease study. 2019 Oct 22;12 1 107.2019 Oct 22;12 1 107. Open 7. Massimiliano Salati, Marina Cesaretti, Matteo Macchia et al. Epidemiological Overview of Hodgkin Lymphoma across the Mediterranean Basin. 2014 Jul 1;6 1):e2014048.2014 Jul 1;6 1):e2014048. Open 8. Satish Shanbhag, Richard F Ambinder. Hodgkin lymphoma: A review and update on recent progress. 2018 Mar;68 2 116 132.2018 Mar;68 2 116 132. Open 9. Collins GP, Parker AN, Pocock C et al. Guideline on the management of primary resistant and relapsed classical Hodgkin lymphoma. Br J Haematol. 2014 Jan;164 1 39 52. Open 10. Hoppe RT, Advani RH, Ai WZ et al. NCCN Guidelines Insights: Hodgkin Lymphoma, Version 1.2018. J Natl Compr Canc Netw. 2018 Mar;16 3 245 254. Open 11. Satish Shanbhag, Richard F Ambinder. Hodgkin lymphoma: A review and update on recent progress. CA Cancer J Clin. 2018 Mar;68 2 116 132. Open 12. Massimiliano Salati, Marina Cesaretti, Matteo Macchia et al. Epidemiological Overview of Hodgkin Lymphoma across the Mediterranean Basin. Mediterr J Hematol Infect Dis. 2014 Jul 1;6 1):e2014048. Open https://web.pathway.md/diseases/recbCbpohsw9AgeZT 4/5 6/24/23, 12:45 PM Hodgkin's lymphoma Pathway 13. Pareen Shenoy, Alison Maggioncalda, Neha Malik et al. Incidence patterns and outcomes for hodgkin lymphoma patients in the United States. Adv Hematol. 2011;2011 725219. Open 14. David Perez-Callejo, Lorea Zurutuza, Ana Royuela et al. 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C Buske, M Hutchings, M Ladetto et al. ESMO Consensus Conference on malignant lymphoma: general perspectives and recommendations for the clinical management of the elderly patient with malignant lymphoma. Ann Oncol. 2018 Mar 1;29 3 544 562. Open 20. David J Straus, Monika D ugosz-Danecka, Joseph M Connors et al. Brentuximab vedotin with chemotherapy for stage III or IV classical Hodgkin lymphoma ECHELON 1 5-year update of an international, open-label, randomised, phase 3 trial. Lancet Haematol. 2021 Jun;8 6):e410-e421. Open https://web.pathway.md/diseases/recbCbpohsw9AgeZT 5/5