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Inhibitors to Coagulation Factors

  • Book
  • © 1995

Overview

Editors:
  1. Louis M. Aledort

    1. Mount Sinai Medical School, New York, USA

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  2. Leon W. Hoyer

    1. American Red Cross Biomedical Services, Rockville, USA

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  3. Jeanne M. Lusher

    1. Wayne State University School of Medicine and The Children’s Hospital of Michigan, Detroit, USA

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  4. Howard M. Reisner

    1. University of North Carolina at Chapel Hill, Chapel Hill, USA

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  5. Gilbert C. White

    1. University of North Carolina at Chapel Hill, Chapel Hill, USA

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Part of the book series: Advances in Experimental Medicine and Biology (AEMB, volume 386)

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Table of contents (22 chapters)

  1. Front Matter

    Pages I-XV
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  2. Factors VIII, IX and von Willebrand factor, their molecular and antigenic structure:An overview

    1. Front Matter

      Pages 1-1
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    2. Structure and function of Factor VIII

      • Pete Lollar
      Pages 3-17

    3. Molecular etiology of factor VIII deficiency in hemophilia A

      • Stylianos E. Antonarakis, Haig H. Kazazian, Jane Gitschier, Pierre Hutter, Philippe de Moerloose, Michael A. Morris
      Pages 19-34

    4. The incidence of factor VIII inhibitors in patients with severe hemophilia A

      • Leon W. Hoyer
      Pages 35-45

    5. Epitope specificity and functional characterization of factor VIII inhibitors

      • Dorothea Scandella, Craig Kessler, Pamela Esmon, Deborah Hurst, Suzanne Courter, Edward Gomperts et al.
      Pages 47-63

    6. Immunogenetics of the human immune response to factor VIII

      • Howard M. Reisner, Adella Clark, Linda Levin
      Pages 65-78

    7. Factor IX:Molecular structure, epitopes, and mutations associated with inhibitor formation

      • Katherine A. High
      Pages 79-86

    8. Antibodies to von Willebrand factor in von Willebrand disease

      • P. M. Mannucci, A. B. Federici
      Pages 87-92

  3. The molecular basis of the human immune response

    1. Front Matter

      Pages 93-93
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    2. B-cell epitopes:Fact and fiction

      • David C. Benjamin
      Pages 95-108

    3. Antigen analogs as therapeutic agents

      • Jeff Alexander, Jörg Ruppert, Dawne M. Page, Stephen M. Hedrick, Alessandra Franco, Glenn Y. Ishioka et al.
      Pages 109-118

  4. Genetic variation and the immune response

    1. Front Matter

      Pages 119-119
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    2. T-cell receptors in immune responses

      • M. A. Robinson
      Pages 121-132

    3. Immunoglobulin heavy chain variable region gene usage in human autoimmune diseases

      • Virginia Pascual, J. Donald Capra
      Pages 133-139

  5. Treatmnt of inhibitors

    1. Front Matter

      Pages 141-142
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    2. Porcine factor VIII therapy in patients with factor VIII inhibitors

      • Charles Hay, Jay N. Lozier
      Pages 143-151

    3. Clinical presentation and management of patients with circulating anticoagulants

      • Alison Moliterno, William R. Bell
      Pages 153-155

    4. The function of factor VIIa in hemophilia A: An hypothesis

      • Yale Nemerson
      Pages 157-161

    5. Clinical update on the use of recombinant factor VII

      • Steven Glazer, Ulla Hedner, Jorn F. Falch
      Pages 163-174
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Keywords

About this book

"For the blood is the life . . . . "(Deut. 12 :23) " . . . because the blood, in its value as life, makes atonement" (Lev. 17: 11) HemoPhilia is a rare disease, severe hemophilia rarer still, yet the written history of hemophilia extends back over a millennium and a half. In the ancient Middle East, blood and life were coupled. Blood was the primary substance necessary for life, given to God in sacrifice and forbidden as a food to mortals by Levitical law. Blood was essential for rites of purification and consecration. But the flow of blood during menstruation or parturition rendered a woman unclean. The circumcision of a male child required 33 days of "blood purification" by the mother. ' Circumcision, the visible reminder of the covenant of Abraham lijith Yahweh, was required of newborn Jewish males. It "connote(d) suitability for participation in what God is doing. "2 Hence, free and uncontrolled bleeding of the male child during circumcision, during the ratification of God's covenant, would be noted with awe and concern by those of the Jewish faith. It should not be surprising that the first genetic counseling offered to families with hemophilia is found in the Babylonian Talmud (compilation of Jewish law dated to about the third century AD) and concerns the necessity for circumcision in families with what we would now call hemophilia.

Editors and Affiliations

  • Mount Sinai Medical School, New York, USA

    Louis M. Aledort

  • American Red Cross Biomedical Services, Rockville, USA

    Leon W. Hoyer

  • Wayne State University School of Medicine and The Children’s Hospital of Michigan, Detroit, USA

    Jeanne M. Lusher

  • University of North Carolina at Chapel Hill, Chapel Hill, USA

    Howard M. Reisner, Gilbert C. White

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