Main description:

This book sheds new light on clinical, biological and therapeutic data on the rare disease Waldenstroem'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 Waldenstroem 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.

Contents:

Preface: Tribute to J Waldenstroem

Part I: Tumor Cells and microenvironment

1. Waldenstroem Macroglobulinaemia: Pathological Features and Diagnostic Assessment

2. Waldenstroem's Macroglobulinemia Immunophenotype

3. Predispositions and Origins of Waldenstrom Macroglobulinemia: Implications from Genetic Analysis

4. Cytogenetics in Waldenstroem Macroglobulinemia (WM)

5. Genetic and Signaling Abnormalities in Waldenstroem's Macroglobulinemia

6. Molecular Pathways in Growth and Survival: Epigenomics

7. The Bone Marrow Microenvironment and Tumor Cells Interactions in Waldenstroem's Macroglobulinemia

8. Waldenstroem's Macroglobulinaemia: Immunosurveillance and the Immune Micro-environment

Part II: Epidemiology and Genetic Predisposition

9. Epidemiology of Waldenstroem Macroglobulinemia

10. Genetic Predisposition to Waldenstroem Macroglobulinemia

11. Immunoglobulin Type M Monoclonal Gammopathy of Undetermined Significance (IgM-MGUS)

Part III Clinical Features

12. Hyperviscosity Syndrome, Cold Agglutinin Hemolytic Anemia, and Cryoglobulinemia

13. Neuropathy in Waldenstroem's Macroglobulinaemia

14. IgM Amyloidosis

15. The Bing-Neel Syndrome

16. Unusual Manifestations of IgM Monoclonal Gammopathies

Part IV: Laboratory Investigations

17. Laboratory Investigations and Findings: Hematological Abnormalities, Biochemical Investigations, Free Light and Heavy Chains

Part V: Response

18. Response Assessment in Waldenstroem's Macroglobulinaemia

Part VI: Prognostic Factors

19. Risk Stratification in Waldenstroem Macroglobulinemia

Part VII: Treatment Options and Recommendations

20. Indications for Treatment of Waldenstroem's Macroglobulinemia

21. Immunotherapy in Waldenstroem's Macroglobulinemia

22. Immunomodulatory Agents and Proteasome Inhibitors in Waldenstroem's Macroglobulinemia

23. Signal Inhibitors in Waldenstroem's Macroglobulinemia

24. High-Dose Therapy and Haemopoietic Stem Cell Transplantation in Waldenstroem's Macroglobulinaemia

25. Long-Term Toxicity of Therapy in Waldenstroem Macroglobulinemia

26. Treatment Recommendations in Waldenstroem Macroglobulinemia