Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome

被引:48
作者
Amoura, Z
Costedoat-Chalumeau, N
Veyradier, AS
Wolf, M
Ghillani-Dalbin, P
Cacoub, P
Meyer, D
Piette, JC
机构
[1] CHU Pitie Salpetriere, Serv Med Interne, F-75651 Paris 13, France
[2] Hop Kremlin Bicetre, INSERM U143, Le Kremlin Bicetre, France
来源
ARTHRITIS AND RHEUMATISM | 2004年 / 50卷 / 10期
关键词
D O I
10.1002/art.20551
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Arterial thrombotic events, thrombocytopenia, and hemolytic anemia with schistocytes may be encountered in the setting of both thrombotic thrombocytopenic purpura (TTP) and primary antiphospholipid syndrome (APS). We report 2 cases of TTP occurring in patients with definite primary APS. We also describe the results of tests for ADAMTS-13 activity in 20 consecutive patients with primary APS, as well as tests for antiphospholipid antibodies in 26 patients who had TTP, severe ADAMTS-13 deficiency, and ADAMTS-13-inhibiting antibodies. In both of the patients with primary APS and TTP, ADAMTS-13 activity was undetectable, and ADAMTS-13-inhibiting antibodies were present. None of the 26 patients with TTP and severe ADAMTS-13 deficiency was positive for the lupus anticoagulant. One of these patients had a low level of anticardiolipin antibodies (22 IgG phospholipid units). In the 20 patients with primary APS, mean ADAMTS-13 activity was 116% (range 44-250%), and no severe deficiency (<5%) was observed. Our findings suggest that primary APS must be added to the list of autoimmune disorders that can be complicated by TTP.
引用
收藏
页码:3260 / 3264
页数:5
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