ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission

被引：216

作者：

Peyvandi, Flora ^{[1
]}

Lavoretano, Silvia ^{[1
]}

Palla, Roberta ^{[1
]}

Feys, Hendrik B. ^{[2
]}

Vanhoorelbeke, Karen ^{[2
]}

Battaglioli, Tullia ^{[1
]}

Valsecchi, Carla ^{[1
]}

Canciani, Maria Teresa ^{[1
]}

Fabris, Fabrizio ^{[3
]}

Zver, Samo ^{[4
]}

Reti, Marienn ^{[5
]}

Mikovic, Danijela ^{[6
]}

Karimi, Mehran ^{[7
]}

Giuffrida, Gaetano ^{[8
]}

Laurenti, Luca ^{[9
]}

Mannucci, Pier Mannuccio ^{[1
]}

机构：

[1] Univ Milan, Angelo Bianchi Bonomi Hemophilia & Thrombosis Ctr, Mangiagalli & Regina Elena Fdn, Luigi Villa Fdn,IRCCS Maggiore Hosp,Dept Med & Me, Milan, Italy

[2] Katholieke Univ Leuven, IRC, Lab Thrombosis Res, Kortrijk, Belgium

[3] Univ Padua, Unit Dept Med & Surg Sci, Internal Med, Padua, Italy

[4] Univ Med Ctr, Dept Haematol, Ljubljana, Slovenia

[5] Natl Med Ctr, Dept Hematol & Stem Cell Transplantat, Budapest, Hungary

[6] Natl Blood Transfus Inst, Emostasis Dept & Hemophilia Ctr, Belgrade, Serbia

[7] Shiraz Univ Med Sci, Nemazee Hosp, Haematol Res Ctr, Haemostasis & Thrombosis Unit, Shiraz, Iran

[8] Univ Catania, Vittorio Emanuele Ferrarotto S Bambino Hosp, Div Hematol & Bone Marrow Transplantat, Catania, Italy

[9] Catholic Univ, Hematol Inst, Rome, Italy

来源：

HAEMATOLOGICA | 2008年 / 93卷 / 02期

关键词：

thrombotic thrombocytopenic purpura; ADAMTS13; von Willebrand factor; risk factors; recurrence;

D O I：

10.3324/haematol.11739

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Background From 20 to 50% of patients who survive an acute episode of the acquired form of thrombotic thrombocytopenic purpura relapse but clinical and laboratory markers of recurrence are not well established. Design and Methods In 109 patients enrolled in an international registry we evaluated, in the frame of a retrospective cohort study, the predictive role of the metalloprotease ADAMTS13 as measured in plasma during remission. Anti-ADAMTS13 antibodies and von Willebrand factor were also evaluated in a smaller number of the same patients. Results Median values of ADAMTS13 activity and antigen were significantly lower in patients with recurrent thrombotic thrombocytopenic purpura than in those with no recurrence (activity: 12% vs. 41%; p=0.007; antigen: 36% vs. 58%; p=0.003). A severe deficiency of ADAMTS13 activity (10% or less) was associated with a higher likelihood of recurrence (odds ratio 2.9; 95% confidence interval 1.3 to 6.8; p=0.01). Anti-ADAMTS13 antibodies were also more prevalent in patients with recurrent thrombotic thrombocytopenic purpura (odds ratio 3.1; 95% confidence interval 1.4 to 7.3; p=0.006). The presence during remission of both severe ADAMTS13 deficiency and anti-ADAMTS13 antibodies increased the likelihood of recurrence 3.6 times (95% confidence interval 1.4 to 9.0; p=0.006). The presence of ultra large von Willebrand factor multimers and of associated diseases or conditions did not increase recurrence. Conclusions Survivors of an acute episode of acquired thrombotic thrombocytopenic purpura with severely reduced levels of ADAMTS13 and/or with anti-ADAMTS13 antibodies during remission have an approximately three-fold greater likelihood of developing another episode of thrombotic thrombocytopenic purpura than patients with higher protease activity and no antibody.

引用

页码：232 / 239

页数：8

共 34 条

[31] ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients [J].