ABSTRACT

Background 1173 Historical perspective 1174 Pathology 1174 Clinical considerations 1175 Presenting symptoms 1175 Making the diagnosis 1175 Natural history 1176 Clinical course 1177 Management 1179 To treat or not to treat? 1179 Radiation therapy 1180 Treatment of advanced disease 1180 Specific treatment options 1181

Treatment at recurrence 1186 High-dose treatment with autologous hematopoietic 1187

cell support Allogeneic bone marrow transplantation 1188 Vaccination 1188 General considerations 1189 Follow-up 1189 Evaluation of response 1189 Entry into clinical trials 1189 Prognostic factors 1189 Conclusion 1190 Key points 1190 References 1191

Follicular lymphoma is the second most common histologic subtype of non-Hodgkin lymphoma (NHL) worldwide.1 The World Health Organization (WHO) Lymphoma Classification2 is based on cell of origin and the pathophysiological behavior of the lymphoma. Follicular lymphoma is derived from germinal center B cells and, in terms of gene expression profiling,3 maintains the molecular features of this stage in B cell ontogeny. Occurring with a frequency of approximately 2:100 000 in the Western world,2

its incidence varies with geographic location and amongst different ethnic groups, being seen less often in Asia and developing countries than in Europe and the United States.4 This difference is reflected in the fact that the risk of developing the illness is also lower in first-generation immigrants from China and Japan than for subsequent generations living in the United States.5