Seminars in Hematology
Volume 41, Issue 1 , Pages 1-3 , January 2004

Thrombotic thrombocytopenic purpura: advances in pathophysiology, diagnosis, and treatment—introduction

  • Bernhard Lämmle

      Affiliations

    • Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland
    • Corresponding Author InformationAddress correspondence to Bernhard Lämmle, MD, Central Hematology Laboratory, Inselspital, University Hospital, CH 3010, Bern, Switzerland
    • ,
  • James N. George

      Affiliations

    • Hematology-Oncology Section, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA

References 

  1. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89–93
  2. Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura (Report of 16 cases and review of the literature). Medicine. 1966;45:139–159
  3. Bukowski RM, Hewlett JS, Harris JW, Hoffman GC, Battle JD, Silverblatt E, et al.  Exchange transfusions in the treatment of thrombotic thrombocytopenic purpura. Semin Hematol. 1976;13:219–232
  4. Byrnes JJ, Khurana M. Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med. 1977;297:1386–1389
  5. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, et al.  Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med. 1991;325:393–397
  6. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, et al.  Unusually large plasma factor VIII (von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura). N Engl J Med. 1982;307:1432–1435
  7. Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223–4234
  8. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996;87:4235–4244
  9. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, et al.  Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339:1578–1584
  10. Tsai HM, Lian ECY. Antibodies to von-Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585–1594
  11. Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276:41059–41063
  12. Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, et al.  Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413:488–494
  13. Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002;347:589–600
  14. George JN, Vesely SK. Thrombotic thrombocytopenic purpura (From the bench to the bedside, but not yet to the community). Ann Intern Med. 2003;138:152–153 (editorial)
  15. Furlan M, Lämmle B. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome (The role of von Willebrand factor-cleaving protease). Best Pract Res Clin Haematol. 2001;14:437–454
  16. Vesely SK, George JN, Lämmle B, Studt JD, Alberio L, El-Harake MA, et al.  ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (Relation to presenting features and clinical outcomes in a prospective cohort of 142 patients). Blood. 2003;101:60–68
  17. Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hamolytisch-uramische Syndrome (Bilaterale Nierenrindennekrosen bei akuten erworbenen hamolytischen Anamien). Schweiz Med Wochenschr. 1955;85:905–909
  18. Kojouri K, Vesely S, George JN. Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (Frequency, clinical features, and long-term outcomes). Ann Intern Med. 2001;135:1047–1051

PII: S0037-1963(03)00286-5

doi: 10.1053/j.seminhematol.2003.11.001

Seminars in Hematology
Volume 41, Issue 1 , Pages 1-3 , January 2004

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