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Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease?

Publication ,  Journal Article
Willis, MS; Bandarenko, N
Published in: Semin Thromb Hemost
December 2005

The recurrence of thrombotic thrombocytopenic purpura (TTP) in 20 to 50% of patients who survive their initial episode, is not only a challenge to manage clinically, but brings to light our lack of understanding of the underlying pathophysiology. Recurrence may occur close to apparent hematologic remission or remotely, months later. Given that surveillance of TTP relies on surrogate measures such as hematologic recovery, normalization of serum lactate dehydrogenase (LDH) and resolution of clinical signs, it is plausible that some recurrences are actually a continuum of disease. Standardizing definitions for remission, exacerbation, and relapse are important for consistency of patient management and interpreting the medical literature. When TTP recurs, several adjuncts to therapeutic plasma exchange (TPE) have been used, including splenectomy, vincristine, and rituximab. Although it would seem intuitive that more severe disease would predict a greater likelihood of relapse, the degree of thrombocytopenia at presentation, the persistence of schistocytosis at discontinuation of TPE, the elevation of LDH, and the length of time to attain clinical remission are not reliable indicators of the clinical outcome of any individual TTP patient. Measurement of ADAMTS13 activity and its inhibitor offer new promise for prognostic and pathophysiologic significance; however, more extensive scientific investigation is needed.

Duke Scholars

Published In

Semin Thromb Hemost

DOI

ISSN

0094-6176

Publication Date

December 2005

Volume

31

Issue

6

Start / End Page

700 / 708

Location

United States

Related Subject Headings

  • Vincristine
  • Splenectomy
  • Rituximab
  • Remission Induction
  • Recurrence
  • Purpura, Thrombotic Thrombocytopenic
  • Plasma Exchange
  • L-Lactate Dehydrogenase
  • Humans
  • Cardiovascular System & Hematology
 

Citation

APA
Chicago
ICMJE
MLA
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Willis, M. S., & Bandarenko, N. (2005). Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease? Semin Thromb Hemost, 31(6), 700–708. https://doi.org/10.1055/s-2005-925476
Willis, Monte S., and Nicholas Bandarenko. “Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease?Semin Thromb Hemost 31, no. 6 (December 2005): 700–708. https://doi.org/10.1055/s-2005-925476.
Willis MS, Bandarenko N. Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease? Semin Thromb Hemost. 2005 Dec;31(6):700–8.
Willis, Monte S., and Nicholas Bandarenko. “Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease?Semin Thromb Hemost, vol. 31, no. 6, Dec. 2005, pp. 700–08. Pubmed, doi:10.1055/s-2005-925476.
Willis MS, Bandarenko N. Relapse of thrombotic thrombocytopenic purpura: is it a continuum of disease? Semin Thromb Hemost. 2005 Dec;31(6):700–708.
Journal cover image

Published In

Semin Thromb Hemost

DOI

ISSN

0094-6176

Publication Date

December 2005

Volume

31

Issue

6

Start / End Page

700 / 708

Location

United States

Related Subject Headings

  • Vincristine
  • Splenectomy
  • Rituximab
  • Remission Induction
  • Recurrence
  • Purpura, Thrombotic Thrombocytopenic
  • Plasma Exchange
  • L-Lactate Dehydrogenase
  • Humans
  • Cardiovascular System & Hematology