ABSTRACT

Introduction 1279 Epidemiology 1279 Incidence 1279 Risk factors 1280 Biology 1280 Histology 1281 Clinical considerations 1281 Presentation and staging 1281 Diagnostic evaluation 1281 Prognostic factors 1283 Initial treatment for pediatric Hodgkin lymphoma 1283 Treatment for relapsed Hodgkin lymphoma 1287

Long-term outcomes of Hodgkin lymphoma and 1288 its treatment

Second malignant neoplasms 1288 Cardiac dysfunction 1288 Pulmonary dysfunction 1288 Thyroid disease 1288 Gonadal dysfunction and infertility 1289 Psychosocial problems 1290 Monitoring for adverse long-term outcomes 1290 Conclusions 1291 Key points 1291 References 1291

The first description of Hodgkin lymphoma (HL) was anatomic in nature, in 1832, when Thomas Hodgkin described seven patients with enlarged lymph glands and spleen. This was followed, at the turn of the twentieth century, by histologic descriptions of the now-characteristic multinucleated giant cells by Sternberg in 18981 and Reed in 1902.2 In the 1960s, the clonality of the Reed-Sternberg cell was established3 and in the past decade attention has been focused on understanding the molecular biology of the disease, with respect to immunoglobulin genes, transcription factors, apoptotic pathways, and Epstein-Barr virus (EBV) incorporation.4-7

Therapeutic advances in HL began in 1902 when Pusey reported on the use of radiation therapy.8 This was followed by the use of single-agent chemotherapy with mechlorethamine (nitrogen mustard) in 1946.9 Over the next two decades, research was directed toward the use of combination chemotherapy, leading to the introduction of the four-drug MOPP protocol in 196410 and ABVD in the 1970s.11 Donaldson and colleagues at Stanford introduced the concept of combined modality therapy for pediatric patients using the MOPP backbone and low-dose radiation therapy.12 (See Table 73.4 for acronyms of treatment

protocols.) This was followed both in the medical and pediatric oncology arenas by the rapid development of multimodality, risk-adapted therapies designed to balance efficacy with toxicity, as well as the study of adverse longterm outcomes related to the disease and its treatment.