Table Of Content

Rare Hematological Malignancies Cancer Treatment and Research Steven T. Rosen, M.D., Series Editor Pienta, K.J. (ed.): Diagnosis and Treatment of Genitourinary Malignancies. 1996. ISBN 0-7923-4164-3. Arnold, A.J. (ed.): Endocrine Neoplasms. 1997. ISBN 0-7923-4354-9. Pollock, R.E. (ed.): Surgical Oncology. 1997. ISBN 0-7923-9900-5. Verweij, J., Pinedo, H.M., Suit, H.D. (eds): Soft Tissue Sarcomas: Present Achievements and Future Prospects. 1997. ISBN 0-7923-9913-7. Walterhouse, D.O., Cohn, S. L. (eds): Diagnostic and Therapeutic Advances in Pediatric Oncology. 1997. ISBN 0-7923-9978-1. Mittal, B.B., Purdy, J.A., Ang, K.K. (eds): Radiation Therapy. 1998. ISBN 0-7923-9981-1. Foon, K.A., Muss, H.B. (eds): Biological and Hormonal Therapies of Cancer. 1998. ISBN 0-7923-9997-8. Ozols, R.F. (ed.): Gynecologic Oncology. 1998. ISBN 0-7923-8070-3. Noskin, G. A. (ed.): Management of Infectious Complications in Cancer Patients. 1998. ISBN 0-7923-8150-5. Bennett, C. L. (ed.): Cancer Policy. 1998. ISBN 0-7923-8203-X. Benson, A. B. (ed.): Gastrointestinal Oncology. 1998. ISBN 0-7923-8205-6. Tallman, M.S., Gordon, L.I. (eds): Diagnostic and Therapeutic Advances in Hematologic Malignancies. 1998. ISBN 0-7923-8206-4. von Gunten, C.F. (ed.): Palliative Care and Rehabilitation of Cancer Patients. 1999. ISBN 0-7923-8525-X Burt, R.K., Brush, M.M. (eds): Advances in Allogeneic Hematopoietic Stem Cell Transplantation. 1999. ISBN 0-7923-7714-1. Angelos, P. (ed.): Ethical Issues in Cancer Patient Care 2000. ISBN 0-7923-7726-5. Gradishar, W.J., Wood, W.C. (eds): Advances in Breast Cancer Management. 2000. ISBN 0-7923-7890-3. Sparano, J. A. (ed.): HIV & HTLV-I Associated Malignancies. 2001. ISBN 0-7923-7220-4. Ettinger, D. S. (ed.): Thoracic Oncology. 2001. ISBN 0-7923-7248-4. Bergan, R. C. (ed.): Cancer Chemoprevention. 2001. ISBN 0-7923-7259-X. Raza, A., Mundle, S.D. (eds): Myelodysplastic Syndromes & Secondary Acute Myelogenous Leukemia 2001. ISBN: 0-7923-7396. Talamonti, M. S. (ed.): Liver Directed Therapy for Primary and Metastatic Liver Tumors. 2001. ISBN 0-7923-7523-8. Stack, M.S., Fishman, D.A. (eds): Ovarian Cancer. 2001. ISBN 0-7923-7530-0. Bashey, A., Ball, E.D. (eds): Non-Myeloablative Allogeneic Transplantation. 2002. ISBN 0-7923-7646-3. Leong, S. P.L. (ed.): Atlas of Selective Sentinel Lymphadenectomy for Melanoma, Breast Cancer and Colon Cancer. 2002. ISBN 1-4020-7013-6. Andersson , B., Murray D. (eds): Clinically Relevant Resistance in Cancer Chemotherapy. 2002. ISBN 1-4020-7200-7. Beam, C. (ed.): Biostatistical Applications in Cancer Research. 2002. ISBN 1-4020-7226-0. Brockstein, B., Masters, G. (eds): Head and Neck Cancer. 2003. ISBN 1-4020-7336-4. Frank, D.A. (ed.): Signal Transduction in Cancer. 2003. ISBN 1-4020-7340-2. Figlin, R. A. (ed.): Kidney Cancer. 2003. ISBN 1-4020-7457-3. Kirsch, M.; Black, P. McL. (ed.): Angiogenesis in Brain Tumors. 2003. ISBN 1-4020-7704-1. Keller, E.T., Chung, L.W.K. (eds): The Biology of Skeletal Metastases. 2004. ISBN 1-4020-7749-1. Kumar, R. (ed.): Molecular Targeting and Signal Transduction. 2004. ISBN 1-4020-7822-6. Verweij, J., Pinedo, H.M. (eds): Targeting Treatment of Soft Tissue Sarcomas. 2004. ISBN 1-4020-7808-0. Finn, W.G., Peterson, L.C. (eds.): Hematopathology in Oncology. 2004. ISBN 1-4020-7919-2. Farid, N. (ed.): Molecular Basis of Thyroid Cancer. 2004. ISBN 1-4020-8106-5. Khleif, S. (ed.): Tumor Immunology and Cancer Vaccines. 2004. ISBN 1-4020-8119-7. continued after Index Stephen M. Ansell, MD, PhD Editor Rare Hematological Malignancies Stephen M. Ansell, MD, PhD Associate Professor of Medicine Mayo Clinic, College of Medicine 200 First Street SW Rochester, Minnesota, USA Series Editor: Steven T. Rosen Robert H. Lurie Comprehensive Cancer Center Northwestern University Chicago, IL USA ISBN-13: 978-0-387-73743-0 e-ISBN-13: 978-0-387-73744-7 Library of Congress Control Number: 2007932231 © 2008 Springer Science + Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science + Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. Printed on acid-free paper. 9 8 7 6 5 4 3 2 1 springer.com Preface Advances in diagnostic techniques and therapeutic modalities have brought about significant progress in the management of many hematologic malignancies. For the practicing clinician these changes are occurring at a rapid rate and it is difficult to keep informed of all the new information. Therefore, most physicians focus on the major diagnostic and therapeutic changes in common cancers, and it is often a challenge to stay up to date on the advances in the less common malignancies. Furthermore, it is difficult to find the new information in a timely fashion when seeing patients with an uncommon or unusual hematologic malignancy. In this book, we have assembled data on rare hematologic malignancies to provide a c oncise resource for practicing oncologists. Each chapter provides information on the biology of the various rare hematologic diseases but also focuses on the latest therapeutic advances. Because of the paucity of clinical trials in most of these d iseases, the authors have not only summarized the literature but also provided their own expertise regarding management of patients. We trust that this book will be helpful to physicians seeing patients with uncommon hematologic malignancies, and will also be of benefit to patients by providing the treating physicians with r ecommendations concerning the optimal management of diseases that are uncom- monly seen in their practice. Stephen M. Ansell, MD, PhD Division of Hematology Mayo Clinic v Contents Contributors ................................................................................................... ix 1 Polycythemia Vera and Other Polycythemia Syndromes ..................... 1 Richard T. Silver 2 Primary Myelofi brosis ............................................................................. 29 Ayalew Tefferi 3 Essential Thrombocythemia.................................................................... 51 Guido Finazzi 4 Chronic Eosinophilic Leukemia/ Hypereosinophilic Syndrome .................................................................. 69 Jason Gotlib 5 Chronic Myelomonocytic Leukemia ...................................................... 107 Miloslav Beran 6 The 5q– Syndrome ................................................................................... 133 Aristoteles A.N. Giagounidis and Carlo Aul 7 Rare Acute Leukemias ............................................................................. 149 Xavier Georges Thomas 8 Hairy Cell Leukemia ................................................................................ 193 Paul Timothy Fanta and Alan Saven 9 Waldenstrom’s Macrogloblinemia/ Lymphoplasmacytic Lymphoma............................................................. 211 Steven P. Treon, Evdoxia Hatjiharissi, and Giampaolo Merlini vii viii Contents 10 Rare B-cell Lymphomas: Primary Mediastinal, Intravascular, and Primary Effusion Lymphoma ........................................................ 243 Kerry J. Savage 11 Lymphomatoid Granulomatosis ........................................................... 265 Ryan B. Lundell, Roger H. Weenig, and Lawrence E. Gibson 12 Posttransplant Lymphoproliferative Disorders .................................. 273 Thomas M. Habermann 13 Castleman Disease .................................................................................. 293 Angela Dispenzieri 14 Rare T-Cell Lymphomas........................................................................ 331 Ana Maria Molina and Steven M. Horwitz 15 CD30+ Diseases: Anaplastic Large-Cell Lymphoma and Lymphomatoid Papulosis ............................................................... 349 Peter Borchmann 16 Nodular Lymphocyte Predominant Hodgkin’s Lymphoma ............... 367 Michelle A. Fanale and Anas Younes 17 Langerhans Cell Histiocytosis ............................................................... 383 Karen L. Chang and David S. Snyder 18 Systemic Mastocytosis ........................................................................... 399 Peter Valent Index ................................................................................................................ 421 Contributors Stephen M. Ansell Mayo Clinic College of Medicine, Rochester, MN Carlo Aul St. Johannes Hospital, Duisburg, Germany Milosav Beran University of Texas M.D. Anderson Cancer Center, Houston TX Peter Borchmann University of Cologne, Cologne, Germany Karen L. Chang City of Hope National Medical Center, Duarte, CA Angela Dispenzieri Mayo Clinic College of Medicine, Rochester, MN Michelle A. Fanale University of Texas M.D. Anderson Cancer Center, Houston, TX Paul T. Fanta Scripps Clinic, La Jolla, CA Guido Finazzi Ospedali Riuniti, Bergamo, Italy Aristoteles A.N. Giagounidis St. Johannes Hospital, Duisburg, Germany Lawrence E. Gibson Mayo Clinic College of Medicine, Rochester, MN Jason Gotlib Stanford Cancer Center, Stanford, CA Thomas M. Habermann Mayo Clinic College of Medicine, Rochester, MN ix x Contributors Evdoxia Hatjiharissi Dana Farber Cancer Institute, Boston, MA Steven M. Horwitz Memorial Sloan Kettering Cancer Center, New York, N Y Ryan B. Lundell Mayo Clinic College of Medicine, Rochester, MN Giampaolo Merlini Amyloidosis Center, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo and Department of Biochemistry, University of Pavia, Italy Ana Maria Molina Memorial Sloan Kettering Cancer Center, New York, NY Kerry J. Savage British Columbia Cancer Agency, Vancouver, Canada Alan Saven Scripps Clinic,. La Jolla, CA Richard T. Silver Weill Cornell Medical University, New York, NY David S. Snyder City of Hope National Medical Center, Duarte, CA Ayalew Tefferi Mayo Clinic College of Medicine, Rochester, MN Xavier G. Thomas Edouard Herriot Hospital, Lyon, France Steven P. Treon Dana Farber Cancer Institute, Boston, MA Peter Valent Medical University of Vienna, Vienna, Austria Roger H. Weenig Mayo Clinic College of Medicine, Rochester, MN Anas Younes University of Texas M.D. Anderson Cancer Center, Houston, TX

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Many comprehensive text books have been written about the common hematologic malignancies. However, every practicing hematologist/oncologist or primary care physician intermittently encounters patients with uncommon or rare hematologic malignancies. Information concerning these diseases, and particu
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435 Pages·2008·51.5 MB·English
by  Richard T. Silver (auth.)
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Rare Hematological Malignancies Cancer Treatment and Research Steven T. Rosen, M.D., Series Editor Pienta, K.J. (ed.): Diagnosis and Treatment of Genitourinary Malignancies. 1996. ISBN 0-7923-4164-3. Arnold, A.J. (ed.): Endocrine Neoplasms. 1997. ISBN 0-7923-4354-9. Pollock, R.E. (ed.): Surgical Oncology. 1997. ISBN 0-7923-9900-5. Verweij, J., Pinedo, H.M., Suit, H.D. (eds): Soft Tissue Sarcomas: Present Achievements and Future Prospects. 1997. ISBN 0-7923-9913-7. Walterhouse, D.O., Cohn, S. L. (eds): Diagnostic and Therapeutic Advances in Pediatric Oncology. 1997. ISBN 0-7923-9978-1. Mittal, B.B., Purdy, J.A., Ang, K.K. (eds): Radiation Therapy. 1998. ISBN 0-7923-9981-1. Foon, K.A., Muss, H.B. (eds): Biological and Hormonal Therapies of Cancer. 1998. ISBN 0-7923-9997-8. Ozols, R.F. (ed.): Gynecologic Oncology. 1998. ISBN 0-7923-8070-3. Noskin, G. A. (ed.): Management of Infectious Complications in Cancer Patients. 1998. ISBN 0-7923-8150-5. Bennett, C. L. (ed.): Cancer Policy. 1998. ISBN 0-7923-8203-X. Benson, A. B. (ed.): Gastrointestinal Oncology. 1998. ISBN 0-7923-8205-6. Tallman, M.S., Gordon, L.I. (eds): Diagnostic and Therapeutic Advances in Hematologic Malignancies. 1998. ISBN 0-7923-8206-4. von Gunten, C.F. (ed.): Palliative Care and Rehabilitation of Cancer Patients. 1999. ISBN 0-7923-8525-X Burt, R.K., Brush, M.M. (eds): Advances in Allogeneic Hematopoietic Stem Cell Transplantation. 1999. ISBN 0-7923-7714-1. Angelos, P. (ed.): Ethical Issues in Cancer Patient Care 2000. ISBN 0-7923-7726-5. Gradishar, W.J., Wood, W.C. (eds): Advances in Breast Cancer Management. 2000. ISBN 0-7923-7890-3. Sparano, J. A. (ed.): HIV & HTLV-I Associated Malignancies. 2001. ISBN 0-7923-7220-4. Ettinger, D. S. (ed.): Thoracic Oncology. 2001. ISBN 0-7923-7248-4. Bergan, R. C. (ed.): Cancer Chemoprevention. 2001. ISBN 0-7923-7259-X. Raza, A., Mundle, S.D. (eds): Myelodysplastic Syndromes & Secondary Acute Myelogenous Leukemia 2001. ISBN: 0-7923-7396. Talamonti, M. S. (ed.): Liver Directed Therapy for Primary and Metastatic Liver Tumors. 2001. ISBN 0-7923-7523-8. Stack, M.S., Fishman, D.A. (eds): Ovarian Cancer. 2001. ISBN 0-7923-7530-0. Bashey, A., Ball, E.D. (eds): Non-Myeloablative Allogeneic Transplantation. 2002. ISBN 0-7923-7646-3. Leong, S. P.L. (ed.): Atlas of Selective Sentinel Lymphadenectomy for Melanoma, Breast Cancer and Colon Cancer. 2002. ISBN 1-4020-7013-6. Andersson , B., Murray D. (eds): Clinically Relevant Resistance in Cancer Chemotherapy. 2002. ISBN 1-4020-7200-7. Beam, C. (ed.): Biostatistical Applications in Cancer Research. 2002. ISBN 1-4020-7226-0. Brockstein, B., Masters, G. (eds): Head and Neck Cancer. 2003. ISBN 1-4020-7336-4. Frank, D.A. (ed.): Signal Transduction in Cancer. 2003. ISBN 1-4020-7340-2. Figlin, R. A. (ed.): Kidney Cancer. 2003. ISBN 1-4020-7457-3. Kirsch, M.; Black, P. McL. (ed.): Angiogenesis in Brain Tumors. 2003. ISBN 1-4020-7704-1. Keller, E.T., Chung, L.W.K. (eds): The Biology of Skeletal Metastases. 2004. ISBN 1-4020-7749-1. Kumar, R. (ed.): Molecular Targeting and Signal Transduction. 2004. ISBN 1-4020-7822-6. Verweij, J., Pinedo, H.M. (eds): Targeting Treatment of Soft Tissue Sarcomas. 2004. ISBN 1-4020-7808-0. Finn, W.G., Peterson, L.C. (eds.): Hematopathology in Oncology. 2004. ISBN 1-4020-7919-2. Farid, N. (ed.): Molecular Basis of Thyroid Cancer. 2004. ISBN 1-4020-8106-5. Khleif, S. (ed.): Tumor Immunology and Cancer Vaccines. 2004. ISBN 1-4020-8119-7. continued after Index Stephen M. Ansell, MD, PhD Editor Rare Hematological Malignancies Stephen M. Ansell, MD, PhD Associate Professor of Medicine Mayo Clinic, College of Medicine 200 First Street SW Rochester, Minnesota, USA Series Editor: Steven T. Rosen Robert H. Lurie Comprehensive Cancer Center Northwestern University Chicago, IL USA ISBN-13: 978-0-387-73743-0 e-ISBN-13: 978-0-387-73744-7 Library of Congress Control Number: 2007932231 © 2008 Springer Science + Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science + Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. Printed on acid-free paper. 9 8 7 6 5 4 3 2 1 springer.com Preface Advances in diagnostic techniques and therapeutic modalities have brought about significant progress in the management of many hematologic malignancies. For the practicing clinician these changes are occurring at a rapid rate and it is difficult to keep informed of all the new information. Therefore, most physicians focus on the major diagnostic and therapeutic changes in common cancers, and it is often a challenge to stay up to date on the advances in the less common malignancies. Furthermore, it is difficult to find the new information in a timely fashion when seeing patients with an uncommon or unusual hematologic malignancy. In this book, we have assembled data on rare hematologic malignancies to provide a c oncise resource for practicing oncologists. Each chapter provides information on the biology of the various rare hematologic diseases but also focuses on the latest therapeutic advances. Because of the paucity of clinical trials in most of these d iseases, the authors have not only summarized the literature but also provided their own expertise regarding management of patients. We trust that this book will be helpful to physicians seeing patients with uncommon hematologic malignancies, and will also be of benefit to patients by providing the treating physicians with r ecommendations concerning the optimal management of diseases that are uncom- monly seen in their practice. Stephen M. Ansell, MD, PhD Division of Hematology Mayo Clinic v Contents Contributors ................................................................................................... ix 1 Polycythemia Vera and Other Polycythemia Syndromes ..................... 1 Richard T. Silver 2 Primary Myelofi brosis ............................................................................. 29 Ayalew Tefferi 3 Essential Thrombocythemia.................................................................... 51 Guido Finazzi 4 Chronic Eosinophilic Leukemia/ Hypereosinophilic Syndrome .................................................................. 69 Jason Gotlib 5 Chronic Myelomonocytic Leukemia ...................................................... 107 Miloslav Beran 6 The 5q– Syndrome ................................................................................... 133 Aristoteles A.N. Giagounidis and Carlo Aul 7 Rare Acute Leukemias ............................................................................. 149 Xavier Georges Thomas 8 Hairy Cell Leukemia ................................................................................ 193 Paul Timothy Fanta and Alan Saven 9 Waldenstrom’s Macrogloblinemia/ Lymphoplasmacytic Lymphoma............................................................. 211 Steven P. Treon, Evdoxia Hatjiharissi, and Giampaolo Merlini vii viii Contents 10 Rare B-cell Lymphomas: Primary Mediastinal, Intravascular, and Primary Effusion Lymphoma ........................................................ 243 Kerry J. Savage 11 Lymphomatoid Granulomatosis ........................................................... 265 Ryan B. Lundell, Roger H. Weenig, and Lawrence E. Gibson 12 Posttransplant Lymphoproliferative Disorders .................................. 273 Thomas M. Habermann 13 Castleman Disease .................................................................................. 293 Angela Dispenzieri 14 Rare T-Cell Lymphomas........................................................................ 331 Ana Maria Molina and Steven M. Horwitz 15 CD30+ Diseases: Anaplastic Large-Cell Lymphoma and Lymphomatoid Papulosis ............................................................... 349 Peter Borchmann 16 Nodular Lymphocyte Predominant Hodgkin’s Lymphoma ............... 367 Michelle A. Fanale and Anas Younes 17 Langerhans Cell Histiocytosis ............................................................... 383 Karen L. Chang and David S. Snyder 18 Systemic Mastocytosis ........................................................................... 399 Peter Valent Index ................................................................................................................ 421 Contributors Stephen M. Ansell Mayo Clinic College of Medicine, Rochester, MN Carlo Aul St. Johannes Hospital, Duisburg, Germany Milosav Beran University of Texas M.D. Anderson Cancer Center, Houston TX Peter Borchmann University of Cologne, Cologne, Germany Karen L. Chang City of Hope National Medical Center, Duarte, CA Angela Dispenzieri Mayo Clinic College of Medicine, Rochester, MN Michelle A. Fanale University of Texas M.D. Anderson Cancer Center, Houston, TX Paul T. Fanta Scripps Clinic, La Jolla, CA Guido Finazzi Ospedali Riuniti, Bergamo, Italy Aristoteles A.N. Giagounidis St. Johannes Hospital, Duisburg, Germany Lawrence E. Gibson Mayo Clinic College of Medicine, Rochester, MN Jason Gotlib Stanford Cancer Center, Stanford, CA Thomas M. Habermann Mayo Clinic College of Medicine, Rochester, MN ix x Contributors Evdoxia Hatjiharissi Dana Farber Cancer Institute, Boston, MA Steven M. Horwitz Memorial Sloan Kettering Cancer Center, New York, N Y Ryan B. Lundell Mayo Clinic College of Medicine, Rochester, MN Giampaolo Merlini Amyloidosis Center, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo and Department of Biochemistry, University of Pavia, Italy Ana Maria Molina Memorial Sloan Kettering Cancer Center, New York, NY Kerry J. Savage British Columbia Cancer Agency, Vancouver, Canada Alan Saven Scripps Clinic,. La Jolla, CA Richard T. Silver Weill Cornell Medical University, New York, NY David S. Snyder City of Hope National Medical Center, Duarte, CA Ayalew Tefferi Mayo Clinic College of Medicine, Rochester, MN Xavier G. Thomas Edouard Herriot Hospital, Lyon, France Steven P. Treon Dana Farber Cancer Institute, Boston, MA Peter Valent Medical University of Vienna, Vienna, Austria Roger H. Weenig Mayo Clinic College of Medicine, Rochester, MN Anas Younes University of Texas M.D. Anderson Cancer Center, Houston, TX

Description:
Many comprehensive text books have been written about the common hematologic malignancies. However, every practicing hematologist/oncologist or primary care physician intermittently encounters patients with uncommon or rare hematologic malignancies. Information concerning these diseases, and particu
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