Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Thrombotic Thrombocytopenic Purpura (TTP) Differential Diagnoses

  • Author: Theodore Wun, MD, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
 
Updated: Jun 20, 2016
 
 

Diagnostic Considerations

To make an accurate diagnosis, the clinician must recognize the similarity between thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS). In addition to HUS, the differential diagnosis also includes immune thrombocytopenic purpura (ITP) and disseminated intravascular coagulation (DIC), two entities with very different modes of therapy (see the image below).

Differential diagnosis of thrombotic thrombocytopeDifferential diagnosis of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome.

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Theodore Wun, MD, FACP Professor of Medicine, Professor of Pathology and Laboratory Medicine, University of California Davis School of Medicine; Chief of Hematology/Oncology, Program Director, Veterans Affairs Northern California Health Care System; Medical Director, University of California Davis CCRC

Theodore Wun, MD, FACP is a member of the following medical societies: American Association of Blood Banks, American Society for Blood and Marrow Transplantation, American College of Physicians, American Federation for Medical Research, American Society of Hematology, SWOG

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Srikanth Nagalla, MBBS, MS, FACP Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

Srikanth Nagalla, MBBS, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Nothing to disclose.

Acknowledgements

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

References
  1. Tsai HM. Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update. Hematol Oncol Clin North Am. 2013 Jun. 27(3):565-84. [Medline].

  2. Ferrari S, Mudde GC, Rieger M, Veyradier A, Kremer Hovinga JA, Scheiflinger F. IgG-subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2009 Aug 11. [Medline].

  3. Mariotte E, Azoulay E, Galicier L, Rondeau E, Zouiti F, Boisseau P, et al. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016 May. 3 (5):e237-45. [Medline].

  4. Lowes R. FDA Okays Blood Plasma Product for Clotting Disorders. Medscape Medical News. January 17, 2013. Available at http://www.medscape.com/viewarticle/777822. Accessed: January 31, 2013.

  5. Vesely SK, George JN, Lammle B, 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 Jul 1. 102(1):60-8. [Medline].

  6. Sauna ZE, Okunji C, Hunt RC, et al. Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. PLoS One. 2009 Aug 5. 4(8):e6506. [Medline]. [Full Text].

  7. Miller DP, Kaye JA, Shea K, Ziyadeh N, Cali C, Black C, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004 Mar. 15 (2):208-15. [Medline].

  8. Scully M, Thomas M, Underwood M, Watson H, Langley K, Camilleri RS, et al. Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood. 2014 Jul 10. 124(2):211-9. [Medline].

  9. Bouw MC, Dors N, van Ommen H, Ramakers-van Woerden NL. Thrombotic thrombocytopenic purpura in childhood. Pediatr Blood Cancer. 2009 Jun 18. 53(4):537-542. [Medline].

  10. Wu N, Liu J, Yang S, Kellett ET, Cataland SR, Li H, et al. Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura. Transfusion. 2014 Jun 23. [Medline].

  11. Coppo P, Veyradier A. Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura. Presse Med. 2012 Mar. 41(3 Pt 2):e163-76. [Medline].

  12. [Guideline] Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug. 158 (3):323-35. [Medline]. [Full Text].

  13. Shah N, Rutherford C, Matevosyan K, Shen YM, Sarode R. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol. 2013 Nov. 163 (4):514-9. [Medline].

  14. Connell NT, Cheves T, Sweeney JD. Effect of ADAMTS13 activity turnaround time on plasma utilization for suspected thrombotic thrombocytopenic purpura. Transfusion. 2015 Oct 12. [Medline].

  15. Duffy SM, Coyle TE. Platelet transfusions and bleeding complications associated with plasma exchange catheter placement in patients with presumed thrombotic thrombocytopenic purpura. J Clin Apher. 2013 May 30. [Medline].

  16. Marn Pernat A, Buturovic-Ponikvar J, Kovac J, et al. Membrane plasma exchange for the treatment of thrombotic thrombocytopenic purpura. Ther Apher Dial. 2009 Aug. 13(4):318-21. [Medline].

  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 Feb. 53(2):259-72. [Medline].

  18. Fakhouri F, Vernant JP, Veyradier A, et al. Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood. 2005 Sep 15. 106(6):1932-7. [Medline].

  19. Froissart A, Buffet M, Veyradier A, Poullin P, Provôt F, Malot S, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med. 2012 Jan. 40(1):104-11. [Medline].

  20. Becerra E, Scully MA, Leandro MJ, Heelas EO, Westwood JP, De La Torre I, et al. Effect of rituximab on B-cell phenotype and serum B-cell activating factor levels in patients with Thrombotic Thrombocytopenic Purpura. Clin Exp Immunol. 2014 Oct 22. [Medline].

  21. Patriquin CJ, Thomas MR, Dutt T, McGuckin S, Blombery PA, Cranfield T, et al. Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura. Br J Haematol. 2016 Mar 24. [Medline].

  22. Zhou A, Mehta RS, Smith RE. Outcomes of platelet transfusion in patients with thrombotic thrombocytopenic purpura: a retrospective case series study. Ann Hematol. 2014 Oct 7. [Medline].

  23. Gringauz I, Carmel-Neiderman NN, Mangel T, Portnoy O, Segal G, Goren I. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy. Case Rep Hematol. 2016. 2016:1568586. [Medline]. [Full Text].

  24. Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knöbl P, Wu H, et al. Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura. N Engl J Med. 2016 Feb 11. 374 (6):511-22. [Medline]. [Full Text].

  25. Phase III Trial With Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura (HERCULES). ClinicalTrials.gov. Available at https://clinicaltrials.gov/ct2/show/NCT02553317. March 17, 2016; Accessed: May 13, 2016.

  26. Jhaveri KD, Scheuer A, Cohen J, Gordon B. Treatment of refractory thrombotic thrombocytopenic purpura using multimodality therapy including splenectomy and cyclosporine. Transfus Apher Sci. 2009 Aug. 41(1):19-22. [Medline].

  27. Scully M, McDonald V, Cavenagh J, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011 Aug 18. 118(7):1746-53. [Medline].

  28. Shumak KH, Rock GA, Nair RC. Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura. Canadian Apheresis Group. Ann Intern Med. 1995 Apr 15. 122(8):569-72. [Medline].

  29. Roriz M, Landais M, Desprez J, et al; French Thrombotic Microangiopathies Reference Center. Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura. Medicine (Baltimore). 2015 Oct. 94 (42):e1598. [Medline]. [Full Text].

  30. Sikka P, Chopra S, Aggarwal N, Suri V, Chandrasekaran A. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy. Asian J Transfus Sci. 2013 Jan. 7(1):79-80. [Medline]. [Full Text].

Previous
Next
 
Differential diagnosis of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.