Symposium in Immunology III: Humoral Immunodeficiencies (Primary and Secondary) PDF
Preview Symposium in Immunology III: Humoral Immunodeficiencies (Primary and Secondary)
M. M. Eibl C. Huber H. H. Peter U. Wahn (Eds.) Symposium in Immunology III Humoral Immunodeficiencies (Primary and Secondary) With 28 Figures and 27 Tables Springer-Verlag Berlin Heidelberg New York London Paris Tokyo Hong Kong Barcelona Budapest Prof. Dr. MARTHA M. EIBL lnstitut fur lmmunologie der Universitat Wien Borschkegasse 8a A-I090Wien Austria Prof. Dr. CHRISTOPH HUBER Abteilung fur Hamatologie Johannes Gutenberg Universitat Langenbeckstr. 1 D-55131 Mainz FRG Prof. Dr. HANS H. PETER Abteilung fUr Rheumatologie und Klinische lmmunologie Medizinische Universitatsklinik Hugstetter Str. 55 D-79106 Freiburg FRG Prof. Dr. ULRICHWAHN Padiatrische Pneumologie und lmmunologie Universitatsklinikum Rudolf Virchow Standort Charlottenburg Heubnerweg 6 D-14059 Berlin FRG ISBN -13: 978-3-540-57126-1 e -ISBN -13: 978-3-642-78438-5 DOl: 10.1007/978-3-642-78438-5 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, speci fically the rights of translation, reprinting, reuse of illustration, recitation, broadcasting, reproduction, on microfilms or in other ways, and storage in data banks. Duplication of this publication or parts thereof is only permitted under the provisions of the German Copyright Law of September 9, 1965, in its current version, and a copyright fee must always be paid. Violations fall under the prosecution act of the German Copyright Law. © Springer-Verlag Berlin Heidelberg 1994 The use of general descriptive names, trade names, trade marks, etc. in this publication, even if the former are not espe cially identified, is not to be taken as a sign that such names, as understood by the Trade Marks and Merchandise Marks Act may accordingly be used freely by anyone. Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof con tained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceut icalliterature. Typesetting: Mitterweger Werksatz, Plankstadt 27/313015432 1 0 - Printed on acid-free paper Contents Basic Biology Introductory Remarks G. VICARI .................... . 3 B Cell Development in the Mouse: Scenarios for Defects Expressed in the B Cell Lineage F. MELCHERS, U. GRAWUNDER, D. HAASNER, H. KARASUYAMA, L. REININGER, and A. ROLINK ...................... 5 Factors and Receptors Involved in the Selection of B Lymphocytes Responding to T-Dependent Antigen J. GORDON ...................................... 13 Antibody Activities of Human Monoclonal Immunoglobulins M. SELIGMANN, J.-c. BROUET, and X. MARIETTE ............. 25 Immune Regulation, Biology, Pathology Human Types 1 and 2 T Helper Subsets: Functional Properties, Role in Disease and Regulation of Development S. ROMAGNANI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 35 The Use of Targeted Mutations in ES Cells to Create Novel Immunodeficient Mouse Models F. W. ALT and J. CHEN ............................... 45 BTK, A Novel Protein Tyrosine Kinase Mutated in X-Linked Agammaglobulinemia C. 1. E. SMITH, 1. VORECHOVSKY, L. HAMMARSTROM, D. VETRIE, and P. SIDERAS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 Clinics Clinical and Immunological Aspects of Common Variable Immunodeficiency F. AIUTI, G. LUZI, E. SCALA, A. OLIVA, R. Rosso, and F. PANDOLFI 71 VI Contents Clinical Relevance of Subclass Deficiency Diseases L. A. HANSON, M. HAHN-ZORIC, V. FRIMAN, K. THEMAN, J. BJORKANDER, A. H. LUCAS, and A. T. NAGAO ............. 77 Different Categories of Common Variable Immunodeficiency J. FARRANT and A. D. B. WEBSTER ...................... 91 Immunoregulatory Effects of Immunoglobulin D A. R. AMIN, C. D. SWENSON, and G. J. THORBECKE ........... 103 Mucosal Immunity in Immunodeficiency P. BRANDTZAEG and D. E. NILS SEN ... 119 Strategies in the Diagnosis and Treatment of Immunodeficiency Diseases R. A. THOMPSON 161 Therapy Immunodeficiency with Normal or Increased Immunoglobulin M F. S. ROSEN ...................................... 171 Modulation of the Inflammatory Response by Human Immunoglobulin A M. M. EIBL and H. M. WOLF ................... . 179 Intravenous Immunoglobulins in the Treatment of Recurrent Seizures A. G. UGAZIO, M. DUSE, E. MENEGATI, A. PLEBANI, L. D. NOTARANGELO, and S. TIBERTI ..................... 197 Role of Cytokines in the Regulation of Malignant B Cell Proliferation C. PESCHEL, T. FLOHR, P. TRAUTMANN, and C. HUBER .......... 211 Subject Index ..................................... 219 List of Contributors F. NUTI Department of Allergy and Clinical Immunology, University of Rome "La Sapienza", 1-00185 Rome, Italy F. W. ALT Howard Hughes Medical Institute, Children's Hospital, Department of Genetics, Harvard Medical School, and The Center for Blood Research, 300 Longwood Avenue, Boston, Massachusetts 02115, USA A.R.AMIN Department of Pathology, NYU Medical Center, New York, NY 10016, USA J. BJORKANDER Asthma and Allergy Research Centre, University of Goteborg, S-41346 Goteborg, Sweden P. BRANDTZAEG Laboratory for Immunohistochemistry and Immunopathology (LIIPAT), Institute of Pathology, The National Hospital, Rikshospitalet, N-0027 Oslo, Norway J.-c. BROUET Department of Immunohematology and Laboratory of Immunopathology of the Insti tut.e of Hematology, Hopital St. Louis, F-75475 Paris, France J. CHEN Howard Hughes Medical Institute, Children's Hospital, Department of Genetics, Harvard Medical School, and The Center for Blood Research, 300 Longwood Avenue, Boston, Massachusetts 02115, USA M. DUSE Department of Paediatrics, University of Brescia, 1-25123 Brescia, Italy M. M. EIBL Institute of Immunology, University of Vienna, Borschkegasse 8a, A-I090 Vienna, Austria J. FARRANT Immunodeficiency Diseases Research Group, Clinical Research Centre, Watford Road, Harrow HAl 3UJ, u.K. T.FLOHR Division of Hematology, III. Department of Internal Medicine, Johannes Gutenberg University, Medical Center, Langenbeckstr. 1, D-55131 Mainz, FRG V. FRIMAN Department of Infectious Diseases, University of Goteborg, S-41346 Goteborg, Sweden VIII List of Contributors J. GORDON Department of Immunology, The Medical School, Vincent Drive, Birmingham B15 2TT, United Kingdom U. GRAWUNDER Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland D. HAASNER Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland M. HAHN-ZoRlc Department of Clinical Immunology, University of Goteborg, Guldhetsgatan 10 A, S-41346 Goteborg, Sweden L. HAMMARSTROM Center for BioTechnology, Karolinska Institute, NOVUM, S-14157 Huddinge and Department of Clinical Immunology, Karolinska Institute at Huddinge Hospital, S-14186 Huddinge, Sweden L. A. HANSON Department of Clinical Immunology, University of Goteborg, Guldhetsgatan 10 A, S-41346 Goteborg, Sweden C. HUBER Division of Hematology, III. Department of Internal Medicine, Johannes Gutenberg University, Medical Center, Langenbeckstr. 1, D-55131 Mainz, FRG H. KARASUYAMA Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland A. H. LUCAS Children's Hospital Oakland Research Institute, Oakland, CA, USA G. LUZI Department of Allergy and Clinical Immunology, University of Rome "La Sapienza", 1-00185 Rome, Italy x. MARlETTE Department of Immunohematology and Laboratory of Immunopathology of the Institute of Hematology, Hopital St. Louis, F-75475 Paris, France F. MELCHERS Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland E. MENEGATI Division of Neurology and Child Psychiatry, Hospital of Brescia, 1-25123-Brescia, Italy A. T. NAGAO Department of Clinical Immunology, University of Goteborg, Guldhetsgatan 10 A, S-41346 Goteborg, Sweden D. E. NILSSEN Department of Internal Medicine A, University of Oslo, The National Hospital, Rikshospitalet, N-0027 Oslo, Norway L. D. NOTARANGELO Department of Paediatrics, University of Brescia, 1-25123-Brescia, Italy List of Contributors IX A. OLIVA Department of Allergy and Clinical Immunology, University of Rome "La Sapienza" , 1-00185 Rome, Italy F. PANDOLFI Chair of Clinical Methodology, Catholic University, 1-00185 Rome, Italy C. PESCHEL Division of Hematology, III. Department of Internal Medicine, Johannes Gutenberg University, Medical Center, Langenbeckstr. 1, D-55131 Mainz, FRG A. PLEBANI Department of Paediatrics, University of Brescia, 1-25123 Brescia, Italy L. REININGER Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland A. ROLINK Basel Institute for Immunology, Postfach, CH-4005 Basel, Switzerland S. ROMAGNANI Division of Clinical Immunology and Allergy, University of Florence, Istituto di Clinica Medica 3, Viale Morgagni 85,1-50134 Firenze, Italy F. S. ROSEN Harvard Medical School, Center for Blood Research, 800 Huntington Avenue, Boston, MA 02115, USA R. Rosso Department of Allergy and Clinical Immunology, University of Rome "La Sapienza", 1-00185 Rome, Italy E. SCALA Department of Allergy and Clinical Immunology, University of Rome "La Sapienza", 1-00185 Rome, Italy M. SELIGMANN Department of Immunohematology and Laboratory of Immunopathology of the Institute of Hematology, Hopital St. Louis, F-75475 Paris, France P. SIDERAS Center for BioTechnology, Karolinska Institute, NOVUM, S-14157 Huddinge and Department of Cell and Molecular Biology, Umea University, S-90187 Umea, Sweden C. I. E. SMITH Center for BioTechnology, Karolinska Institute, NOVUM, S-14157 Huddinge, and Department of Clinical Immunology, Karolinska Institute at Huddinge Hospital, S-14186 Huddinge, Sweden C. D. SWENSON Department of Pathology, NYU Medical Center, New York, NY 10016, USA K. THEMAN Asthma and Allergy Research Centre, University of Goteborg, S-41346 Goteborg, Sweden X List of Contributors R. A. THOMPSON Regional Department of Immunology, East Birmingham Hospital, Birmingham B9 5SS, United Kingdom G. J. THORBECKE Department of Pathology, NYU Medical Center, New York, NY 10016, USA S. TrnERTI Division of Neurology and Child Psychiatry, Hospital of Brescia, 1-25123 Brescia, Italy P. TRAUTMANN Division of Hematology, III. Department of Internal Medicine, Johannes Gutenberg University, Medical Center, Langenbeckstr. 1, D-55131 Mainz, FRG A. G. UGAZIO Department of Paediatrics, University of Brescia, 1-25123 Brescia, Italy D. VETRIE Division of Medical and Molecular Genetics, UMDS of Guy's and St. Thomas's Hospitals, Guy's Tower, London SE1 9RT, United Kindom G. VICARI Istituto Superiore di Sanita, Viale Regina Elena 299,1-00161 Roma, Italy I. VORECHOVSKY Center for BioTechnology, Karolinska Institute, NOVUM, S-14157 Huddinge and Department of Clinical Immunology, Karolinska Institute at Huddinge Hospital, S-14186 Huddinge, Sweden A. D. B. WEBSTER Immunodeficiency Diseases Research Group, Clinical Research Centre, Watford Road, Harrow HAl 3UJ, United Kingdom H.M. WOLF Institute of Immunology, University of Vienna, Borschkegasse 8a, A-1090 Vienna, Austria Basic Biology
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