Thrombotic Thrombocytopenia Purpura (TTP): “Who Are You Calling a FAT RN?”

  • Joshua Faucher
  • Colin G. KaideEmail author


TTP is a type of thrombotic microangiopathy characterized by low platelets and evidence of a microangiopathic hemolytic anemia which produces platelet plugs and resultant red cell debris (called schistocytes). The underlying cause can be idiopathic or it can result from acquired antibodies to a protein called ADAMTS13 which serves to cleave von Willebrand multimers. TTP may also cause neurological abnormalities and less commonly renal failure. Definitive treatment is with plasmapheresis. It can cause a waxing and waning disease pattern. Untreated, the mortality is over 80%.


TTP Thrombotic thrombocytopenia purpura Microangiopathic hemolytic anemia Thrombocytopenia Plasmapheresis Thrombotic microangiopathy 


Disclosure Statement

Joshua Faucher: No disclosures.

Colin Kaide: Callibra, Inc.-Discharge 123 medical software company. Medical Advisory Board Portola Pharmaceuticals. I have no relationship with a commercial company that has a direct financial interest in subject matter or materials discussed in article or with a company making a competing product.


  1. 1.
    Kappler S, Ronan-Bentle S, Graham A. Thrombotic Microangiopathies (TTP, HUS, HELLP). Hematol Oncol Clin North Am. 2017;31:1081–103.CrossRefGoogle Scholar
  2. 2.
    Reese JA, Muthurajah DS, Hovinga JAK, Vesely SK, Terrell DR, George JN. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features. Pediatr Blood Cancer. 2013;60:1676–82.CrossRefGoogle Scholar
  3. 3.
    Terrell DR, Vesely SK, Hovinga JAK, Lämmle B, George JN. Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes. Am J Hematol. 2010;85:844–7.CrossRefGoogle Scholar
  4. 4.
    Koyfman A, Brém E, Chiang VW. Thrombotic thrombocytopenic purpura. Pediatr Emerg Care. 2011;27:1085–8.CrossRefGoogle Scholar
  5. 5.
    George JN. The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS registry, 1989–2007. Kidney Int. 2009; Scholar
  6. 6.
    Tsai H-M. Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura. Kidney Int. 2009; Scholar
  7. 7.
    Furlan M, Robles R, Galbusera M, et al. von Willebrand factor–cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic–uremic syndrome. N Engl J Med. 1998;339:1578–84.CrossRefGoogle Scholar
  8. 8.
    Nokes T, George JN, Vesely SK, Awab A. Pulmonary involvement in patients with thrombotic thrombocytopenic purpura. Eur J Haematol. 2013;92:156–63.CrossRefGoogle Scholar
  9. 9.
    Sadler JE. Pathophysiology of thrombotic thrombocytopenic purpura. Blood. 2017;130(10):1181–8. Scholar
  10. 10.
    Zakarija A, Kwaan HC, Moake JL, et al. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989–2008). Kidney Int. 2009; Scholar
  11. 11.
    Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura. Medicine. 1966;45:139–60.CrossRefGoogle Scholar
  12. 12.
    Page EE, Hovinga JAK, Terrell DR, Vesely SK, George JN. Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Adv. 2017;1:590–600.CrossRefGoogle Scholar
  13. 13.
    George JN. Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why? Transfusion. 2015;55:11–3.CrossRefGoogle Scholar
  14. 14.
    Bendapudi PK, Hurwitz S, Fry A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017;4(4):e157.CrossRefGoogle Scholar
  15. 15.
    Li A, Khalighi PR, Wu Q, Garcia DA. External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment. J Thromb Haemost. 2017;16(1):164–9. Scholar
  16. 16.
    Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med. 1991;325:393–7.CrossRefGoogle Scholar
  17. 17.
    Michael M, Elliott EJ, Craig JC, Ridley G, Hodson EM. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009;53:259–72.CrossRefGoogle Scholar
  18. 18.
    Scully M, Mcdonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, Machin SJ. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011;118:1746–53.CrossRefGoogle Scholar
  19. 19.
    McClain RS, Terrell DR, Vesely SK, George JN. Plasma exchange complications in patients treated for thrombotic thrombocytopenia purpura-hemolytic uremic syndrome: 2011 to 2014. Transfusion. 2014;54:3257–9.CrossRefGoogle Scholar
  20. 20.
    Scully M. Trends in the diagnosis and management of TTP: European perspective. Transfus Apher Sci. 2014;51:11–4.CrossRefGoogle Scholar
  21. 21.
    Swisher KK, Terrell DR, Vesely SK, Hovinga JAK, Lämmle B, George JN. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion. 2009;49:873–87.CrossRefGoogle Scholar
  22. 22.
    Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019; [e-pub].; Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Rush Oak Park HospitalOak ParkUSA
  2. 2.Department of Emergency Medicine, Wexner Medical Center at The Ohio State UniversityColumbusUSA

Personalised recommendations