This book sheds new light on clinical, biological and therapeutic data on the rare disease Waldenström's Macroglobulinemia (WM) with the participation of widely-recognized experts, involved in this field. It represents the efforts of physicians, scientists and patients, all around the world, to better understand and cure this rare disease.

Considerable advances in the diagnosis, treatment indications, response criteria, prognostic factors and treatment options have been made since Dr Jan Waldenström first reported this "new syndrome" 70 years ago. Particularly instrumental in advancing of our understanding of WM have been the eight international workshops devoted to this disease. New, exciting molecular data have recently been reported, allowing us to revisit the oncogenic events leading to WM B-cell proliferation and to use newly available compounds targeting oncogenic pathways.

Autorentext
Véronique Leblond, Professor in Hematology, is the head of the department of Hematology at Pitié Salpêtrière Hospital, Paris, France. She is the chair of the French Cooperative Group on Chronic Lymphocytic Leukemia and Waldenström's Macroglobulinemia: FCG CLL-WM including more than 90 centers in France and Belgium. She is responsible for a rare tumor network (K-VIROGREF) dedicated to the management of transplant recipients with virus-induced tumors and funded by the French national Institute for cancer in 2012. The team historically focuses on chronic B-cell lymphoproliferative disorders, amyloidosis, on ocular and CNS lymphoma and on lymphoproliferations in immunosuppressed patients (HIV, organ-transplanted patients). She is the head of GRECHY, affiliated to the PhD school ED394 Physiology & Physio-pathology. Steven Treon is the Director of the Bing Center for Waldenström's Research and an attending physician for medical oncology, at Dana-Farber Cancer Institute and Brigham and Women's Hospital, in Boston, Massachusetts. He is also an Associate Professor of Medicine at Harvard Medical School in Boston, and is the Chair of the Waldenström's Macroglobulinemia Clinical Trials Group. Dr Treon's main research interests focus on understanding the genetic basis and pathogenesis of Waldenström's macroglobulinemia and the development of therapeutics for this malignancy. Meletios A. Dimopoulos, MD is Professor and Chairman of the Department of Clinical Therapeutics at the University Athens School of Medicine, Athens, Greece. He is a member of numerous scientific societies and has authored more than 650 publications (April 2013) in peer-reviewed journals. Dr. Dimopoulos serves on the Scientific Advisory Boards of the Multiple Myeloma Research Foundation, of the International Myeloma Foundation, of the International Waldenstrom's Macroglobulinemia Foundation and he is a member of the Board of the European Myeloma Network.

Inhalt

Preface: Tribute to J Waldenström.- Part I: Tumor Cells and microenvironment.- Waldenström Macroglobulinaemia: Pathological Features and Diagnostic Assessment.- Waldenström's Macroglobulinemia Immunophenotype.- Predispositions and Origins of Waldenstrom Macroglobulinemia: Implications from Genetic Analysis.- Cytogenetics in Waldenstr<öm Macroglobulinemia (WM).- Genetic and Signaling Abnormalities in Waldenström's Macroglobulinemia.- Molecular Pathways in Growth and Survival: Epigenomics.- The Bone Marrow Microenvironment and Tumor Cells Interactions in Waldenström's Macroglobulinemia.- Waldenström's Macroglobulinaemia: Immunosurveillance and the Immune Micro-environment.- Part II: Epidemiology and Genetic Predisposition.- Epidemiology of Waldenström Macroglobulinemia.- Genetic Predisposition to Waldenström Macroglobulinemia.- Immunoglobulin Type M Monoclonal Gammopathy of Undetermined Significance (IgM-MGUS).- Part III: Clinical Features.- Hyperviscosity Syndrome, Cold Agglutinin Hemolytic Anemia, and Cryoglobulinemia.- Neuropathy in Waldenström's Macroglobulinaemia.- IgM Amyloidosis.- The Bing-Neel Syndrome.- Unusual Manifestations of IgM Monoclonal Gammopathies.- Part IV: Laboratory Investigations.- Laboratory Investigations and Findings: Hematological Abnormalities, Biochemical Investigations, Free Light and Heavy Chains.- Part V: Response.- Response Assessment in Waldenström's Macroglobulinaemia.- Part VI: Prognostic Factors.- Risk Stratification in Waldenström Macroglobulinemia.- Part VII: Treatment Options and Recommendations.- Indications for Treatment of Waldenström's Macroglobulinemia.- Immunotherapy in Waldenström's Macroglobulinemia.- Immunomodulatory Agents and Proteasome Inhibitors in Waldenström's Macroglobulinemia.- Signal Inhibitors in Waldenström's Macroglobulinemia.- High-Dose Therapy and Haemopoietic Stem Cell Transplantation in Waldenström's Macroglobulinaemia.- Long-Term Toxicity of Therapy in Waldenström Macroglobulinemia.- Treatment Recommendations in Waldenström Macroglobulinemia.