Skip to main content

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

  • Chapter
  • First Online:
Case Studies in Emergency Medicine
  • 1492 Accesses

Abstract

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%.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Kappler S, Ronan-Bentle S, Graham A. Thrombotic Microangiopathies (TTP, HUS, HELLP). Hematol Oncol Clin North Am. 2017;31:1081–103.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  4. Koyfman A, Brém E, Chiang VW. Thrombotic thrombocytopenic purpura. Pediatr Emerg Care. 2011;27:1085–8.

    Article  Google Scholar 

  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; https://doi.org/10.1038/ki.2008.622.

    Article  Google Scholar 

  6. Tsai H-M. Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura. Kidney Int. 2009; https://doi.org/10.1038/ki.2008.610.

    Article  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  8. Nokes T, George JN, Vesely SK, Awab A. Pulmonary involvement in patients with thrombotic thrombocytopenic purpura. Eur J Haematol. 2013;92:156–63.

    Article  Google Scholar 

  9. Sadler JE. Pathophysiology of thrombotic thrombocytopenic purpura. Blood. 2017;130(10):1181–8. https://doi.org/10.1182/blood-2017-04-636431.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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; https://doi.org/10.1038/ki.2008.613.

    Article  Google Scholar 

  11. Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura. Medicine. 1966;45:139–60.

    Article  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  13. George JN. Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why? Transfusion. 2015;55:11–3.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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. https://doi.org/10.1111/jth.13882.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  20. Scully M. Trends in the diagnosis and management of TTP: European perspective. Transfus Apher Sci. 2014;51:11–4.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  22. Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019; [e-pub].; https://doi.org/10.1056/NEJMoa1806311.

    Article  CAS  Google Scholar 

Download references

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.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Colin G. Kaide .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Faucher, J., Kaide, C.G. (2020). Thrombotic Thrombocytopenia Purpura (TTP): “Who Are You Calling a FAT RN?” . In: Kaide, C., San Miguel, C. (eds) Case Studies in Emergency Medicine. Springer, Cham. https://doi.org/10.1007/978-3-030-22445-5_58

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-22445-5_58

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-22444-8

  • Online ISBN: 978-3-030-22445-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics