Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Treatment failure in children and adolescents with Hodgkin lymphoma can be divided into three groups:
Primary progressive disease.
Relapse limited to the site(s) of initial involvement (in patients treated with chemotherapy alone).
The presence of B symptoms and extranodal disease at the time of relapse are adverse prognostic features. In one study from the Society for Paediatric Oncology and Haematology (GPOH), patients with an early relapse (defined as occurring...
ABVD: doxorubicin plus bleomycin plus vinblastine plus dacarbazine.
BEACOPP: bleomycin plus etoposide plus doxorubicin plus cyclophosphamide plus vincristine plus procarbazine plus prednisone.
MOPP: mechlorethamine plus vincristine plus procarbazine plus prednisone.
After initial clinical staging for Hodgkin lymphoma (HL), patients with obvious stage III or IV disease, bulky disease (defined as a 10 cm mass or mediastinal disease with a transverse diameter exceeding 33% of the transthoracic diameter), or the presence of B symptoms will require combination chemotherapy with or without additional radiation therapy.
Patients with nonbulky stage IA or IIA disease are considered to have clinical early-stage disease. These patients are candidates for chemotherapy, combined modality therapy, or radiation therapy alone. Staging laparotomy is no longer recommended because it may not alter management and does not enhance ultimate outcome. When chemotherapy alone or combined modality therapy is applied, laparotomy is not required.
In adult HL, the appropriate dose of radiation alone is 25 Gy to 30 Gy to clinically uninvolved sites, and 35 Gy to 44 Gy to regions of initial nodal involvement.[2,3,4,5] These recommendations are often modified in pediatric or advanced-staged adult patients who also receive chemotherapy. Treatment is usually delivered to the neck, chest, and axilla (mantle field) and then to an abdominal field to treat para-aortic nodes and the spleen (splenic pedicle). In some patients, pelvic nodes are treated with a third field. The three fields constitute total nodal radiation therapy. In some cases, the pelvic and para-aortic nodes are treated in a single field called an inverted Y. In patients with a favorable prognosis, treatment of the pelvic lymph nodes is frequently omitted, since fertility can be preserved without affecting relapse-free survival. (For more information on fertility, refer to the Sexuality and Reproductive Issues summary.)
Acute nonlymphocytic leukemia may occur in patients treated with combined modality therapy or with combination chemotherapy alone.[6,7,8] At 10 years following therapy with regimens containing MOPP, the risk of acute myelogenous leukemia (AML) is approximately 3%, with the peak incidence occurring 5 to 9 years after therapy. The risk of acute leukemia at 10 years following therapy with ABVD appears to be less than 1%. A population-based study of more than 35,000 survivors during a 30-year time span identified 217 patients who developed AML; the excess absolute risk is significantly higher (9.9 vs. 4.2 after 1984, P < .001) for older patients (i.e., older than 35 years at diagnosis) versus younger survivors.