Join the 200th Anniversary Celebration

Book Review

Myeloproliferative Disorders

N Engl J Med 2007; 356:2550-2551June 14, 2007

Article

Myeloproliferative Disorders
(Hematologic Malignancies.) Edited by Junia V. Melo and John M. Goldman. 354 pp., illustrated. Berlin, Springer, 2007. $169. ISBN: 978-3-540-34505-3

William Dameshek was my teacher in hematology from 1957 until his death in 1969. He was a pioneer in the treatment of Hodgkin's disease, autoimmune diseases of the blood, and acute leukemia. He was one of the founders of the American Society of Hematology and started, with the support of the medical book publisher Henry Stratton, Blood, the journal of hematology. His 1958 textbook Leukemia (New York: Grune & Stratton), written with Frederick Gunz and featuring beautiful watercolor paintings of leukemia cells by Professor Y. Kawakita of Kumamoto University, Japan, is a classic that continues in its seventh edition under the leadership of the best in the field. Dameshek was an inspiring thinker about the origins of hematologic disorders, and he wrote often about these problems. His two major theoretical breakthroughs — the concept of the myeloproliferative disorders, advanced in 1951, and 30 years later, the notion of the lymphoproliferative diseases — are the bookends of a remarkable career.

Myeloproliferative Disorders, a superb summary of the topic, is a modern synthesis of Dameshek's adventurous thinking. The finding of the same JAK2 mutation in polycythemia vera, myelofibrosis, and essential thrombocytosis stands as the molecular substantiation of the concept of the myeloproliferative disorders. As for chronic myelogenous leukemia (CML), the discovery of the Philadelphia chromosome in 1960, the identification of the consequences of the Philadelphia translocation (the BCR-ABL fusion gene) in 1985, and the introduction into clinical medicine in the 1990s of imatinib (Gleevec, Novartis), an inhibitor of the kinase activity of the ABL protein, are landmark demonstrations of the power of molecular biology to advance not only our understanding of malignant diseases but also the treatment of such diseases.

About two thirds of the chapters in Myeloproliferative Disorders deal with CML, and rightly so, because progress in our understanding of the molecular mechanisms and treatment of this disease has been, without exaggeration, spectacular. Of the book's 18 chapters, 13 discuss CML — its history, its clinical features, the molecular consequences of unregulated intracellular signaling by the tyrosine kinase of ABL that follow the translocation of the ABL gene, the treatment of the disease by inhibitors of the ABL kinase, the results of hematopoietic stem-cell transplantation, new experimental treatments, and ways of monitoring the results of therapy. Other chapters deal with the hypereosinophilic syndrome, polycythemia vera, and essential thrombocytosis.

All the chapters were contributed by experts who have written authoritative, comprehensive, and up-to-date reviews of their assigned topics; citations to publications as recent as 2006 can be found in some chapters. Good editing has achieved clarity of presentation and thought. There is some overlap, especially in the section on CML, but this is not a detriment; each chapter can be read as a stand-alone review. Many useful highlighted tables summarize the data under discussion.

Quibbles in a book review are obligatory, if not inevitable. The artwork in Myeloproliferative Disorders, unfortunately, is spotty and lacks the uniform style expected in an important textbook like this one. John Reilly's chapter on myelofibrosis has excellent illustrations, but many of the figures in other chapters seem to be tiny reproductions of the authors' personal PowerPoint slide collections. Readers may need a magnifying glass to examine the figures in Michael Deininger's review of signal transduction inhibitors. The placement of the chapters is curious; the book begins, appropriately, with a history of CML and then plunges directly into a discussion of the molecular biology of the BCR-ABL translocation. Why does the reader have to wait until chapter 4 to read about the clinical features of CML?

Myeloproliferative Disorders is an important contribution to hematology. It arrives at a time when the study and treatment of these important diseases is at a crossroads, a time when hematologists everywhere have come to expect that the next great leap forward will come soon. For me, this book will sit on the shelf next to the first edition of Dameshek's Leukemia.

Robert Schwartz, M.D.

Citing Articles (1)

Citing Articles

  1. 1

    C. Thomas Caskey. (2010) Using Genetic Diagnosis to Determine Individual Therapeutic Utility. Annual Review of Medicine 61:1, 1-15
    CrossRef