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

被引:18
|
作者
Terrell, Deirdra R.
Motto, David G.
Hovinga, Johanna A. Kremer
Laemmle, Bernhard
George, James N.
Vesely, Sara K. [1 ]
机构
[1] Univ Oklahoma, Hlth Sci Ctr, Dept Biostat & Epidemiol, Coll Publ Hlth, Oklahoma City, OK 73126 USA
基金
瑞士国家科学基金会;
关键词
VON-WILLEBRAND-FACTOR; HEMOLYTIC-UREMIC SYNDROME; FACTOR-CLEAVING PROTEASE; ABO; PLASMA; DISEASE; PROTEOLYSIS; FRETS-VWF73; PHENOTYPE; SURVIVAL;
D O I
10.1111/j.1537-2995.2011.03125.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:2237 / 2243
页数:7
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