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抗磷脂综合征的家族性发病情况。

Familial occurrence of the antiphospholipid syndrome.

作者信息

Matthey F, Walshe K, Mackie I J, Machin S J

机构信息

Department of Haematology, Middlesex Hospital, London.

出版信息

J Clin Pathol. 1989 May;42(5):495-7. doi: 10.1136/jcp.42.5.495.

Abstract

In a family of four the whole spectrum of antiphospholipid and associated antibodies was present but without evidence of connective tissue disease. All four members had anticardiolipin antibodies; two had a confirmed lupus anticoagulant. Thrombocytopenia was severe in one and associated with a high titre of antiplatelet antibody, while another member was found to have a positive antiglobulin test. One member also had a low protein C concentration while two had decreased concentration of protein S. Factors that predispose to these antibodies may be environmental as well as genetic. In view of the well known association of spontaneous thrombotic events with some of these antibodies the prognosis for the family members must be guarded.

摘要

在一个四口之家中,存在抗磷脂抗体及相关抗体的整个谱系,但无结缔组织病的证据。所有四名家庭成员均有抗心磷脂抗体;两人确诊有狼疮抗凝物。其中一人血小板减少严重,且与高滴度抗血小板抗体相关,而另一名家庭成员抗球蛋白试验呈阳性。一名家庭成员蛋白C浓度也较低,两名家庭成员蛋白S浓度降低。易引发这些抗体的因素可能是环境因素以及遗传因素。鉴于自发性血栓形成事件与其中一些抗体之间存在众所周知的关联,该家庭成员的预后必须谨慎对待。

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本文引用的文献

1
Familial association of the lupus anticoagulant.狼疮抗凝物的家族性关联。
Br J Haematol. 1980 May;45(1):89-96. doi: 10.1111/j.1365-2141.1980.tb03814.x.
7
Familial lupus anticoagulants.家族性狼疮抗凝物。
Br J Haematol. 1987 Nov;67(3):359-63. doi: 10.1111/j.1365-2141.1987.tb02358.x.
8
Syndrome of the black swan.黑天鹅综合征
Br J Rheumatol. 1987 Oct;26(5):324-6. doi: 10.1093/rheumatology/26.5.324.

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