Blood group O and black race are independent risk factors for thrombotic thrombocytopenic purpura associated with severe ADAMTS13 deficiency

Transfusion. 2011 Oct;51(10):2237-43. doi: 10.1111/j.1537-2995.2011.03125.x. Epub 2011 Apr 6.

Abstract

Background: It has been postulated that blood group O subjects may be partially protected against thrombotic thrombocytopenic purpura (TTP) because they have lower plasma levels of von Willebrand factor.

Study design and methods: The Oklahoma TTP Registry enrolled 301 consecutive patients from November 13, 1995 (when systematic ADAMTS13 measurements began), through 2009; 281 (93%) patients had ADAMTS13 measurements. Patients were designated as having severe ADAMTS13 deficiency when the activity measurement by either method was less than 10%. ABO blood group was determined in all 281 patients. The observed frequency of blood group O was compared to the expected frequency. The association between severe ADAMTS13 deficiency and blood group, race, sex, and age were analyzed by logistic regression.

Results: The frequency of blood group O was unexpectedly and significantly greater than the race-ethnicity-adjusted expected frequency in 65 patients with severe ADAMTS13 deficiency (60.0% vs. 47.4%, p = 0.042) but not in 216 patients without severe ADAMTS13 deficiency (44.9% vs. 46.5%, p = 0.639). Blood group O and race-ethnicity were independently associated with severe ADAMTS13 deficiency among patients with TTP. The probability for severe ADAMTS13 deficiency was 45.8% with O and 32.1% with non-O blood groups for black patients and 24.1% with O and 15.1% with non-O blood groups for white patients.

Conclusion: Among patients with TTP and severe ADAMTS13 deficiency the relative frequency of patients with blood group O was greater than expected, suggesting that blood group O may be a risk factor for TTP associated with severe ADAMTS13 deficiency.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ABO Blood-Group System*
  • ADAM Proteins / deficiency*
  • ADAMTS13 Protein
  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Asian
  • Black or African American*
  • Female
  • Hispanic or Latino
  • Humans
  • Indians, North American
  • Male
  • Middle Aged
  • Oklahoma / epidemiology
  • Prospective Studies
  • Purpura, Thrombotic Thrombocytopenic / blood*
  • Purpura, Thrombotic Thrombocytopenic / ethnology*
  • Risk Factors
  • Sex Factors
  • White People
  • Young Adult

Substances

  • ABO Blood-Group System
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human