eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Thrombotic Thrombocytopenic Purpura: Follow-up

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
Coauthor(s): 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
Contributor Information and Disclosures

Updated: Aug 27, 2009

Follow-up

Further Inpatient Care

  • Patients with thrombotic thrombocytopenic purpura (TTP) should remain hospitalized until at least a partial response, such as resolution of altered mental status, improved platelet count, and reduced LDH with stable hemoglobin, is achieved.
  • In some patients, intravenous access will become a concern.
    • Large-bore dual-lumen apheresis catheters are now readily available and many are now placed by interventional radiology services.
    • Patients and/or a caregiver can be instructed in the proper care of these catheters.

Further Outpatient Care

  • The necessary outpatient follow-up for patients with TTP and HUS who have entered a complete or partial response is not well defined.
    • Anecdotal reports of increased relapse rates upon abrupt cessation of plasma exchange have resulted in many apheresis services tapering off plasma exchange over the course of 2-3 weeks.
    • However, this practice has not been validated in any prospective or retrospective analysis.
  • Recommendations are that the patient be seen every week for 2 weeks and, if stable, every 2 weeks for a month.
    • During this time, weekly determination of a complete blood count and LDH are performed.
    • If the platelet count drops or the LDH starts to rise, another course of 5 plasma exchanges is reinstituted.
    • If the patient remains stable for a month, the frequency of the follow-up is decreased.
  • The relapse rate is 13-36%, and recurrences as many as 9 years later have been reported.

Transfer

  • Transfer to a facility able to perform apheresis therapy is part of the initial management of these patients.

Complications

  • If patients recover from the acute episode of TTP or HUS, generally, no long-term complications occur.
  • Complications can be divided into disease-related and treatment-related.
    • Disease-related complications are rare. Persistent neurologic abnormalities can occur after otherwise successful treatment of TTP. Abnormalities may result from actual stroke. Persistent renal impairment to the point of requiring dialysis is rare, although mild renal impairment may persist for weeks to months.
    • Treatment-related complications include fluid overload or allergic reactions from plasma infusion. Apheresis catheters can become thrombosed or infected. During the apheresis, hypotension can occur. Paresthesias are related to hypocalcemia from the anticoagulant acid-citrate dextrose (ACD) most commonly used in apheresis procedures; however, this is transient. Long-term complications include the small risk of a blood-borne infection.

Prognosis

  • The overall response rate to plasma exchange is 75-90%.
  • The early mortality rate is 10-20%.
  • Long-term survival is largely dependent on the presence or absence of serious underlying comorbidities such as cancer, HIV infection, or solid organ transplantation. In the authors' series of 126 patients, the estimated 10-year survival rate of patients without comorbid conditions was 82%, compared to a survival rate of 50% if comorbid conditions were present.
  • A clinical severity score, incorporating the presence or absence of neurologic symptoms, creatinine, platelet count, and hemoglobin, was shown to be predictive of 30-day mortality in the authors' retrospective analysis. The absence of fever and a higher creatinine level was associated with a higher rate of relapse. However, upon further analysis of a larger cohort of patients (as yet unpublished), these factors are no longer predictive.

Miscellaneous

Medicolegal Pitfalls

  • To make an accurate diagnosis, the similarity between TTP and HUS must be recognized.
  • The differential diagnosis includes idiopathic thrombocytopenic purpura (ITP) and DIC, 2 entities with very different modes of therapy (see Image 1).
    Differential diagnosis of thrombotic thrombocytop...

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

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    Differential diagnosis of thrombotic thrombocytop...

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

  • Diagnosis must be considered early and, if entertained, plasma infusion and exchange instituted as soon as possible. Most successful suits are due to failure to consider the diagnosis and delays in appropriate initiation of plasma infusion and exchange.

Special Concerns

  • Diagnosing TTP and HUS during pregnancy can be very difficult because the differential includes not only DIC but also preeclampsia/eclampsia and the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets). In general, TTP is more common in the first 2 trimesters, HELLP is more common during the last trimester, and HUS is more common postpartum.
 


More on Thrombotic Thrombocytopenic Purpura

Overview: Thrombotic Thrombocytopenic Purpura
Differential Diagnoses & Workup: Thrombotic Thrombocytopenic Purpura
Treatment & Medication: Thrombotic Thrombocytopenic Purpura
Follow-up: Thrombotic Thrombocytopenic Purpura
Multimedia: Thrombotic Thrombocytopenic Purpura
References
Further Reading
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References

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

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

  3. 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. Aug 11 2009;[Medline].

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

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

  6. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. Aug 8 1991;325(6):398-403. [Medline].

  7. 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. Sep 15 2005;106(6):1932-7. [Medline].

  8. 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. Nov 26 1998;339(22):1578-84. [Medline].

  9. Lara PN, Coe TL, Zhou H, et al. Improved survival with plasma exchange in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Am J Med. Dec 1999;107(6):573-9. [Medline].

  10. Lau DH, Wun T. Early manifestation of thrombotic thrombocytopenic purpura. Am J Med. Nov 1993;95(5):544-5. [Medline].

  11. Moake JL. Haemolytic-uraemic syndrome: basic science. Lancet. Feb 12 1994;343(8894):393-7. [Medline].

  12. Neild GH. Haemolytic-uraemic syndrome in practice. [published erratum appears in Lancet 1994 Feb 26;343(8896):552]. Lancet. Feb 12 1994;343(8894):398-401. [Medline].

  13. Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. Aug 8 1991;325(6):393-7. [Medline].

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

  15. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. Nov 26 1998;339(22):1585-94. [Medline].

  16. 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. Jul 1 2003;102(1):60-8. [Medline].

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Further Reading

Related eMedicine Topics

Clinical Trials
 National Guideline Clearinghouse

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Keywords

thrombotic thrombocytopenic purpura,  TTP, thrombocytopenia, thrombosis, thrombophilia, hemolytic-uremic syndrome, HUS, microangiopathic hemolytic anemia, von Willebrand factor, vWF, platelet count, bland thrombi, Moschowitz syndrome, petechiae, paralysis, coma, ADAMST13

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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 College of Physicians, American Federation for Medical Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Southwest Oncology Group
Disclosure: Nothing to disclose.

Coauthor(s)

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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
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, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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