Hodgkin lymphoma

• Published in: Critical reviews in oncology/hematology 2013-02, Vol.85 (2), p.216-237
• Main Authors: Gobbi, Paolo G, Ferreri, Andrés J.M, Ponzoni, Maurilio, Levis, Alessandro
• Format: Article
• Language: English
• Subjects: ABVD; BEACOPP; Biological and medical sciences; Epstein–Barr virus; Hematologic and hematopoietic diseases; Hematology, Oncology and Palliative Medicine; Hodgkin Disease - diagnosis; Hodgkin Disease - epidemiology; Hodgkin Disease - therapy; Hodgkin lymphoma; Humans; Incidence; Infectious diseases; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Medical sciences; Neoplasm Staging; PET; Prognosis; Risk Factors; Viral diseases
• ISSN: 1040-8428; 1879-0461
• DOI: 10.1016/j.critrevonc.2012.07.002

Abstract Hodgkin lymphoma (HL) is a curable malignancy which shows a bimodal curve in incidence in economically developed countries; there is a putative association with Epstein–Barr virus. The WHO 2008 classification schema recognises two histological types of HL: the nodular lymphocyte predominant and the “classic” HL. The latter encompasses four entities: nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich. Most patients with HL present with asymptomatic superficial lymphadenopathy. The commonest sites of disease are the cervical, supraclavicular and mediastinal lymph nodes, while sub-diaphragmatic presentations and bone marrow and hepatic involvement are less common. Splenic involvement is usually concomitant with hepatic disease and systemic symptoms; extranodal presentations are quite rare. Systemic symptoms are present in ∼35% of cases. The stage of disease is defined according to the Ann Arbor staging system or its Cotswolds variant, and staging work-up includes physical examination, chest X-rays, chest and abdominal CT scan, and bone marrow biopsy.18 FDG-PET (18 fluordeoxyglucose positron emission tomography) plays a central role in staging, response assessment and prognosis definition. Classic HL usually spreads by contiguity within the lymphatic tissue network, with a late extension to adjacent and distant viscera. Mortality from HL has been progressively decreasing, as confirmed by the most recent 5-year survival figure of 81%. The list of putative prognostic factors in HL has been increasing, but most factors still require prospective validation. Some of these variables are used to stratify early-stage disease into “favourable” and “unfavourable” categories, with “unfavourable early-stage” being intermediate between “favourable early-stage” and “advanced-stage”. ABVD (adriamycin(doxorubicin), bleomycin, vinblastine, dacarbazine) combination chemotherapy followed by involved-field irradiation is the standard treatment for patients with early-stage HL, with a 5-year OS >95%. Several trials assessing less intensive approaches for patients with favourable early-stage HL are ongoing. More intensified combinations, such as the BEACOPP (bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine (Oncovin), procarbazine, prednisone) regimen, are being investigated, usually in patients with unfavourable early-stage HL and interim PET+. ABVD is the standard chemotherapy treatment also for patients with advanced disease. A