Patients with localized and disseminated tumors have reduced but measurable levels of ADAMTS-13 (von Willebrand factor cleaving protease)

Pier Mannuccio Mannucci, Mehran Karimi, Ahmad Mosalaei, Maria Teresa Canciani, Flora Peyvandi

Research output: Contribution to journalArticle

45 Citations (Scopus)

Abstract

Background and Objectives. Patients with disseminated malignancies have been noted to have a deficiency of von Willebrand factor (VWF) cleaving protease, ADAMTS-13. The very low or undetectable plasma levels of this protease are said to be similar to those found in patients with thrombotic thrombocytopenic purpura (TTP). This observation, which challenges the paradigm that severe ADAMTS-13 deficiency is a specific diagnostic marker for TTP, remains so far unconfirmed. Design and Methods. We measured the protease and VWF antigen (VWF:Ag) in parallel in 49 Iranian patients with solid tumors, which in 29 cases were localized (stages I and II) and in 20 disseminated (stage IV). Forty-nine healthy individuals matched with cases for sex, age and smoking habits were taken as controls. Results. Patients with disseminated tumors had lower mean plasma levels of ADAMTS-13 than those with localized tumors, but these differences did not reach the level of statistical significance (p=0.059). However, in no patient was the level of ADAMTS-13 below 18% of normal, at variance with previous findings of lower or unmeasurable levels (

Original languageEnglish
Pages (from-to)454-458
Number of pages5
JournalHaematologica
Volume88
Issue number4
Publication statusPublished - Apr 1 2003

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Thrombotic Thrombocytopenic Purpura
Neoplasms
Peptide Hydrolases
von Willebrand Factor
Habits
Smoking
ADAMTS13 Protein
Deficiency Von willebrand factor
Von Willebrand antigen

Keywords

  • ADAMTS-13
  • Thrombotic thrombocytopenic purpura
  • Tumors
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology

Cite this

Patients with localized and disseminated tumors have reduced but measurable levels of ADAMTS-13 (von Willebrand factor cleaving protease). / Mannucci, Pier Mannuccio; Karimi, Mehran; Mosalaei, Ahmad; Canciani, Maria Teresa; Peyvandi, Flora.

In: Haematologica, Vol. 88, No. 4, 01.04.2003, p. 454-458.

Research output: Contribution to journalArticle

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AU - Mannucci, Pier Mannuccio

AU - Karimi, Mehran

AU - Mosalaei, Ahmad

AU - Canciani, Maria Teresa

AU - Peyvandi, Flora

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N2 - Background and Objectives. Patients with disseminated malignancies have been noted to have a deficiency of von Willebrand factor (VWF) cleaving protease, ADAMTS-13. The very low or undetectable plasma levels of this protease are said to be similar to those found in patients with thrombotic thrombocytopenic purpura (TTP). This observation, which challenges the paradigm that severe ADAMTS-13 deficiency is a specific diagnostic marker for TTP, remains so far unconfirmed. Design and Methods. We measured the protease and VWF antigen (VWF:Ag) in parallel in 49 Iranian patients with solid tumors, which in 29 cases were localized (stages I and II) and in 20 disseminated (stage IV). Forty-nine healthy individuals matched with cases for sex, age and smoking habits were taken as controls. Results. Patients with disseminated tumors had lower mean plasma levels of ADAMTS-13 than those with localized tumors, but these differences did not reach the level of statistical significance (p=0.059). However, in no patient was the level of ADAMTS-13 below 18% of normal, at variance with previous findings of lower or unmeasurable levels (

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