This article appears in the following Annals of Oncology issue: ESMO Clinical Recommendations [View the issue table of contents]
ESMO clinical recommendations |
Hodgkin's lymphoma: ESMO Clinical Recommendations for diagnosis, treatment and follow-up
1 Department of Internal Medicine I, University Hospital Köln and German Hodgkin Study Group (GHSG), Köln
2 Department of Medicine III, University Hospital Grosshadern, LMU Munich, Germany
* Correspondence to: ESMO Guidelines Working Group, ESMO Head Office, Via L. Taddei 4, CH-6962 Viganello-Lugano, Switzerland; E-mail: clinicalrecommendations{at}esmo.org
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incidence |
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 Top incidence diagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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The crude incidence of Hodgkin lymphoma (HL) in the European Union is 2.2, the mortality is 0.7/100 000 cases/year.
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diagnosis |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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Pathological diagnosis should be made according to the World Health Organization classification from a surgical specimen/excisional lymph node biopsy whenever possible providing sufficient material for fresh frozen and formalin-fixed samples.
Classical Hodgkin lymphoma (cHL) includes nodular sclerosing, mixed cellularity, lymphocyte-rich and lymphocyte-depleted subtypes and represents about 95% of all HL cases. It is distinguished from nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), which accounts for about 5% of all HL cases.
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staging and risk assessment |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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Chest X-ray and a computed tomography (CT) scan of neck, chest and abdomen are mandatory as well as bone marrow aspiration and histology.
A positron emission tomography (PET) scan may be considered according to the revised response criteria.
Staging laparotomy is not recommended [II, A].
Full blood cell count, erythrocyte sedimentation rate (ESR) and blood chemistry including C-reactive protein, alkaline phosphatase, lactatdehydrogenase, liver enzymes and albumin are obligatory [II–III, A].
Staging is carried out according to the Ann Arbor system in consideration of B-symptoms and the risk factors listed in Table 1. Treatment is chosen according to the categories shown in Table 1 [II–III, A].
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treatment plan |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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cHL, limited stage
Two cycles of adriamycine/bleomycin/vinblastine/dacarbazine (ABVD) are used in combination with 30 Gy involved-field radiotherapy [I, A]. In young adults, chemotherapy-only based regimens result in slightly higher relapse rates but potentially lower long-term toxicity.
cHL, intermediate stage
Four cycles of ABVD are used in combination with 30 Gy involved-field radiotherapy [I, A].
cHL, advanced stage
By default, patients up to age 60 years are treated with either eight cycles of ABVD or eight cycles of bleomycin/etoposide/adriamycine/cyclophospamide/vincristine/procarbazine/prednisone in escalated dosage (BEACOPPescalated) followed by 30 Gy radiotherapy of residual lymphoma larger than 1.5 cm [I–II, A].
Patients older than 60 years should undergo the ABVD chemotherapy due to higher toxicity in this age group.
relapsed cHL patients
In first relapse, salvage regimens like the standard dexamethasone/high-dose ara-C/cisplatin (DHAP) protocol or the recently established ifosphamide/gemcitabine/vinorelbine/dexamethasone (IGEV) protocol, each followed by high-dose chemotherapy and autologous stem cell transplantation can be regarded as the treatment of choice [I, A].
Reduced intensity conditioning allogeneic stem cell transplantation (RIC-allo) should be considered in young, chemosensitive patients in good general condition relapsing after high-dose chemotherapy with autologous stem cell transplantation [II–III, B].
For palliative setting gemcitabine-based chemotherapy can achieve acceptable remission rates, a satisfying quality of life and prolonged survival. Novel single agents and/or regional radiotherapy may also be considered.
NLPHL, stage IA without risk factors
Thirty Gy involved-field radiotherapy alone is the standard treatment for patients in this category [III, A].
NLPHL, other stages
NLPHL treatment is identical to cHL in all stages except for stage IA without risk factors.
relapsed NLPHL patients
A localised NLPHL relapse should be treated with rituximab alone [III, B].
NLPHL patients with a more advanced relapse require more aggressive salvage therapy in combination with rituximab.
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response evaluation |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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Response evaluation should be done after four cycles and after completion of chemotherapy or chemotherapy/radiotherapy. Physical examination, laboratory analysis and CT scans are mandatory. In studies in advanced stage patients, early PET scan after two or three cycles identified poor risk patients. Based on early PET scan further therapy might be adapted to the patient's risk profile [II–III, B]. After completed treatment, positive PET scans may reveal persistent disease activity but false positive PET scan must be excluded.
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follow-up |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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History, physical examination and laboratory analysis including full blood cell count, ESR and blood chemistry should be performed every 3 months for the first year, every 6 months until the fourth year and once a year thereafter [V, D].
Additional evaluation of thyroid function (thyroid-stimulating hormone) after radiation of the neck should be carried out at 1, 2 and at least 5 years [III, A].
CT scans and previously pathologic radiographic tests are performed once to confirm remission status. Afterwards they are indicated if suspicious clinical symptoms occur.
Cancer screening should be conducted regularly due to the risk of secondary malignancies.
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note |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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Levels of evidence [I–V] and grades of recommendation [A–D] as used by the American Society of Clinical Oncology are given in square brackets. Statements without grading were considered justified standard clinical practice by the experts and the ESMO faculty.
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footnotes |
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Approved by the ESMO Guidelines Working Group: February 2002, last update December 2008. This publication supercedes the previously published version—Ann Oncol 2008; 19 (Suppl 2): ii65–ii66.
Conflict of interest: the authors have reported no conflicts of interest.
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references |
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Topincidencediagnosisstaging and risk assessmenttreatment planresponse evaluationfollow-upnotereferences |
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5. Schmitz N, Pfistner B, Sextro M, et al. Aggressive conventional chemotherapy compared with high-dose chemotherapy with autologous haemopoietic stem-cell transplantation for relapsed chemosensitive Hodgkin's disease: a randomised trial. Lancet (2002) 359:2065–2071.[CrossRef][Web of Science][Medline]
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9. Schulz H, Rehwald U, Morschhauser F, et al. Rituximab in relapsed lymphocyte-predominant Hodgkin lymphoma: long-term results of a phase 2 trial by the German Hodgkin Lymphoma Study Group (GHSG). Blood (2008) 111:109–111.
10. Dann EJ, Bar-Shalom R, Tamir A, et al. Risk-adapted BEACOPP regimen can reduce the cumulative dose of chemotherapy for standard and high-risk Hodgkin lymphoma with no impairment of outcome. Blood (2007) 109:905–909.
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