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Hematological Malignancies Hodgkin’s Lymphoma in Adolescents and Young Adults Laura Rodriguez, MD 1 and Angela Punnett, MD 2 1. Research Fellow, Division of Haematology/Oncology, Department of Paediatrics, SickKids Hospital and University of Toronto; 2. Fellowship Program Training Director, Division of Haematology/Oncology, Department of Paediatrics, SickKids Hospital and University of Toronto, Toronto, Canada Abstract Adolescents and young adults (AYAs) are defined as those individuals between the ages of 15 and 39 years. Hodgkin’s lymphoma (HL) is the most common cancer observed in AYAs. Over the last two decades, significant improvements in both survival from HL and the reduction of therapy-related late effects have resulted from the work of collaborative study groups in pediatric and adult domains. The adolescent and young adult (AYA) population falls between these domains. AYA patients are in a critical developmental transition, with significant psychosocial challenges that may impact on the outcome of the primary treatment as well as on the medical care and surveillance of long-term sequelae in survivors. This article will examine available literature regarding outcomes for HL in the AYA population, identifying issues unique to this group, therapeutic options, and specific concerns in follow-up. Keywords Hodgkin’s lymphoma, therapy, late effects, adolescents and young adults, second malignancies Disclosure: The authors have no conflicts of interest to declare. Received: August 22, 2012 Accepted: September 11, 2012 Citation: Oncology & Hematology Review, 2012;8(2):116–22 Correspondence: Angela Punnett, MD, Division of Haematology/Oncology, SickKids Hospital, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada. E: angela.punnett@sickkids.ca Adolescents and young adults (AYAs) are defined as those individuals between the ages of 15 and 39 years. 1 AYAs with cancer are considered a vulnerable group, in large part due to psychosocial challenges in this population, including lack of insurance, low rates of clinical trial enrollment, issues pertaining to independence, and concerns about body image and fertility. The resulting delays in seeking appropriate medical care, delays in diagnosis, and subsequent compliance issues during treatment complicate medical management of cancer among AYAs. 2–5 The most recent US Surveillance, Epidemiology, and End Results data indicate less survival improvement in AYAs with cancer compared with children and older adults. 6 Studies Examining Adolescent and Young Adult Outcomes Studies focusing specifically on AYAs diagnosed with HL are scarce. Reviewing the literature, we found eight studies, and their main characteristics are summarized in Table 1. A UK study analyzed adolescents diagnosed with HL between 1970 and 1997 who received treatment with adult-type protocols. 16 The OS at 5 and 20 years was 90% and 84% for stage I-IIA HL and 72% and 54% respectively for more advanced disease. However, the event-free survival (EFS) at five and 20 years was 59 % and 52 % in stage I–IIA disease, falling to 41 % and 31% in more advanced disease. The authors highlight that EFS is a more reliable measure of treatment efficacy than OS. Hodgkin’s lymphoma (HL) is the most common cancer observed among AYAs. 6 There is a slight overall male predominance in this age group, with a male:female ratio of 1.2. 6–9 Prior history of infection with Epstein–Barr virus (EBV) has been associated with an increased risk of HL. 10 Among AYAs, however, molecular studies show that EBV is related to HL in only a minority of cases and that it is unlikely to play a major etiologic role. 11,12 The most common histological subtype of HL among the adolescent and young adult (AYA) population is nodular sclerosing classical HL, occurring in 50–80 % of patients. 7–9,13,14 Over the past decades, the prognosis of HL Herbertson et al. compared adolescent patients aged 15–19 years and young adult patients aged 20–25 years diagnosed with HL between 1969 and 1998. 17 There was no significant difference in EFS and OS between the two age groups in patients with low and intermediate risk. However, high-risk adolescent patients had a significantly worse disease outcome compared with high-risk young adults, with an EFS at five years of 43.6 % compared with 58.7 % in young adults (p=0.03) and an OS at five years of 66.7 % compared to 84.4 % in young adults (p=0.04). has dramatically improved, 15 but the optimal treatment of AYA patients remains undefined and patients continue to be treated on many different regimens with either pediatric or adult protocols. We will examine available literature regarding outcomes for the AYA population and discuss the therapy-related long-term sequelae. The results of these studies should be interpreted with caution, however, as historical practices no longer considered appropriate, such as staging laparotomy and splenectomy, were employed, as well as older chemotherapy and radiotherapy protocols. 116 © TOUCH BRIEFINGS 2012