Book Review

Pediatric Lymphomas

N Engl J Med 2007; 357:200-201July 12, 2007

Article

Pediatric Lymphomas
(Pediatric Oncology.) Edited by Howard J. Weinstein, Melissa M. Hudson, and Michael P. Link. 291 pp., illustrated. Berlin, Springer, 2007. $129. ISBN: 978-3-540-20356-8

This slim book contains nearly everything a practicing oncologist needs to know about the pathogenesis, diagnosis, therapy, and delayed complications of pediatric lymphomas. The liberal use of color and careful juxtaposition of tables and text in the book invite cover-to-cover reading. The authors include luminaries who developed the field as well as experts who today design clinical trials and carry out correlative molecular investigations. The result is a collection of spirited historical perspectives, practical advice, and unifying principles based on a critical and balanced selection of data.

The explanation of the biology and pathology of non-Hodgkin's lymphoma in one chapter separates disparate tumors into four major groups: Burkitt's lymphoma, diffuse large-B-cell lymphoma, anaplastic large-cell lymphoma, and lymphoblastic lymphoma. Dense text is relieved by original figures and organized tables. Chromosomal rearrangement is explicitly identified as the common pathway that leads to lymphoma by altering the function of a single gene locus or the enhancer sequences of T-cell antigen receptors. The entire chapter is a model of clarity and pedagogy that effectively eliminates confusion.

Four chapters concern the treatment of non-Hodgkin's lymphoma, and two chapters cover lymphoproliferative diseases and T-cell non-Hodgkin's lymphomas that are cutaneous and rare. There is a humbling account of the landmark studies of three drugs that were used to cure half the patients in Uganda who had Burkitt's lymphoma. Descriptions of current studies of drugs that cure most patients in the United States and Europe are in sharp contrast; they involve combinations of many active agents, including anthracycline and corticosteroid, that appear to be important but whose role has not been proved in randomized trials.

There is a succinct account of the identification of the elusive Reed–Sternberg cell as the malignant cell in Hodgkin's lymphoma — a process that took more than 150 years — and its recent characterization as a germinal-center B lymphocyte with antigen-presenting function, work that took another 20 years. More than 30 color photomicrographs enhance the description of the histologic classification of classical Hodgkin's lymphoma and the variant, nodular lymphocyte-predominant Hodgkin's lymphoma. The discussion of the pathogenesis of Hodgkin's lymphoma — which involves a complex interaction among nuclear factor-κB (NF-κB), tumor necrosis factor receptor, Jak–STAT pathways, cytokines, and Epstein–Barr virus (EBV) — would benefit from the addition of figures, since it is difficult for the reader who is unfamiliar with the process to synthesize this topic with text alone.

The sections on the treatment of Hodgkin's lymphoma include information about the consideration of the stage of the disease and demographic factors, including the age and sex of the patient, and they are also richly complemented by a series of tables. These tables define dose and schedules of the MOPP (mechlorethamine, vincristine, procarbazine, and prednisone) and ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) regimens and their derivatives. They also summarize the designs and outcomes of large studies of chemotherapy as the sole treatment versus a combination of judicious radiation and chemotherapy, organized by risk group. The last table is a list of broad recommendations for the treatment of patients with low-risk, intermediate-risk, and high-risk disease. Consideration of the use of surgery followed by observation for the rare patient with localized lymphocyte-predominant Hodgkin's lymphoma is missing from this chapter.

Refractory or resistant disease is a difficult problem in both non-Hodgkin's lymphoma and Hodgkin's lymphoma, especially in patients who have received six or more of the most effective chemotherapeutic agents. Treatment for patients who have a relapse of Hodgkin's lymphoma is discussed in a separate chapter. This discussion is especially useful because most books emphasize first-line therapies. There is a lucid and detailed discussion of reinduction therapies and the rationales for the use of high-dose therapy and stem-cell transplantation, post-transplant salvage, NF-κB proteosome inhibition, and targeted immunotherapy.

The epidemiology of pediatric lymphomas receives attention in many chapters. The section on lymphoproliferative disease includes a concise description of the primary, latent, and reactivated infection with EBV and the normal and abnormal responses of the patient. However, a separate section on epidemiology — including discussions of familial predisposition, environmental factors, and a comprehensive consideration of the role of EBV in all pediatric lymphomas — would have been useful.

Overall, this book is an exceptional, highly accessible resource for oncologists or anyone caring for children and adolescents who are receiving treatment for lymphoma or are in long-term follow-up. There is nothing comparable on the Web or in oncology textbooks. The general principles and the specific pearls, tables, and figures are illuminating and helpful for all who are interested in understanding pediatric lymphomas.

Leslie S. Kersun, M.D., M.S.C.E.
Beverly J. Lange, M.D.
Children's Hospital of Philadelphia, Philadelphia, PA 19104
segall@email.chop.edu