[Clinical and analytical manifestations of primary antiphospholipid antibody syndrome]

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

Series of cases.

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

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).

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.

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.