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[原发性抗磷脂抗体综合征的临床及分析表现]

[Clinical and analytical manifestations of primary antiphospholipid antibody syndrome].

作者信息

Camps García M T, Gutiérrez Rueda A, Guil García M, Perelló-González Moreno I, García Portales R, Belmonte López A, de Rámon Garrido E

机构信息

Servicio de Medicina Interna, Hospital Regional del SAS Carlos Haya, Málaga.

出版信息

An Med Interna. 1993 Dec;10(12):576-82.

PMID:8049321
Abstract

STUDY OBJECTIVE

Description of the clinical and analytical manifestations of 8 patients with Primary Antiphospholipid Syndrome (PAPS).

DESIGN

Series of cases.

SETTING

Patients seen in the Internal Medicine Department of a third level Medical Center in Malaga Province.

PATIENTS AND INTERVENTIONS

We describe the symptoms and signs, as well as the analytical determinations that may be related with autoimmunity in 8 patients diagnosed of PAPS (Harris' Criteria). The antiphospholipid antibodies were determined by a) Biologic false-positive Venereal Disease Research Laboratory (BFP-VDRL), b) Lupus Anticoagulant (LAC): Enlargement of the activated thromboplastin partial time > 6" and Exner test, c) Anticardiolipin antibodies (aCL), IgG (UGPL/ml), IgM (UMPL/ml) by enzyme-linked immunosorbent assay (ELISA).

RESULTS

Four patients were females and four males. The mean age was 35.1 +/- 13.6. None of the patients had criteria of systemic lupus erythematous. The principal clinical manifestations of the PAPS were the venous and arterial thrombotic events in different areas (7 patients); The female patient that didn't have thrombotic event presented thrombocytopenia. Only one patient had 1 abortion. The four females had livedo reticularis (LVR), associated in two of them with arterial hypertension and stroke (Sneddon syndrome). Others manifestations seen, have been, Raynaud's phenomenon, acrocyanosis, migraine, arthritis and myositis. All the patients had IgG aCL, 3 IgM aCL, 7 enlargement of the activated thromboplastin partial time and none presented VRL. Five patient had positive antinuclear antibodies (ANA), but none of them had anti- DNA, hypocomplementemia nor lymphopenia. As far as treatment goes three of the patients are anticoagulated with continuous dicoumarins . The remaining patients keep treatment with platelet antiaggregant, had a satisfactory evolution.

CONCLUSIONS

This group of patients presented venous and arterial thrombotic events such as principal manifestation of the PAPS. The most sensitive test to detect antiphospholipid antibodies were the enlargement of the activated thromboplastin partial time and the aCL. It could be interest the determination of aCL in young people the present thrombotic events without another apparent cause.

摘要

研究目的

描述8例原发性抗磷脂综合征(PAPS)患者的临床及分析表现。

设计

病例系列。

地点

马拉加省一家三级医疗中心内科诊治的患者。

患者与干预措施

我们描述了8例诊断为PAPS(哈里斯标准)患者的症状和体征,以及可能与自身免疫相关的分析测定结果。抗磷脂抗体通过以下方法测定:a)生物学假阳性梅毒血清试验(BFP-VDRL);b)狼疮抗凝物(LAC):活化部分凝血活酶时间延长>6秒及埃克斯纳试验;c)抗心磷脂抗体(aCL),通过酶联免疫吸附测定(ELISA)检测IgG(U GPL/ml)、IgM(U MPL/ml)。

结果

4例女性,4例男性。平均年龄为35.1±13.6岁。所有患者均无系统性红斑狼疮标准。PAPS的主要临床表现为不同部位的静脉和动脉血栓形成事件(7例患者);未发生血栓形成事件的女性患者出现血小板减少。仅1例患者有1次流产。4例女性有网状青斑(LVR),其中2例伴有动脉高血压和中风(斯内登综合征)。其他观察到的表现有雷诺现象、手足发绀、偏头痛、关节炎和肌炎。所有患者均有IgG aCL,3例有IgM aCL,7例活化部分凝血活酶时间延长,均无VDRL阳性。5例患者抗核抗体(ANA)阳性,但均无抗DNA、低补体血症及淋巴细胞减少。就治疗而言,3例患者接受持续双香豆素抗凝治疗。其余患者采用血小板抗聚集剂治疗,病情进展满意。

结论

该组患者以静脉和动脉血栓形成事件为PAPS的主要表现。检测抗磷脂抗体最敏感的试验是活化部分凝血活酶时间延长和aCL。对于无明显其他原因而出现血栓形成事件的年轻人,测定aCL可能有意义。

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