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Hughes'' syndrome (the antiphospholipid syndrome (APS)) presents with recurrent thrombosis, recurrent miscarriage and neurological disease. The major pathogenic mechanism of the syndrome is vascular obstruction (both venous and arterial) due to hypercoagulability. Neurological manifestations are prominent and are often the dominant feature. Headache, migraine and cognitive dysfunction are common while other manifestations such as dementia, epilepsy, chorea, multiple sclerosis (MS), psychiatric disease, transverse myelitis, ocular syndromes, sensorineural hearing loss and movement disorders are also associated with the syndrome. Anticoagulation therapy (either aspirin or oral anticoagulants) can lead to significant improvement. [Copyright &y& Elsevier]

Discusses the use of antiphospholipid antibodies (aPL) as diagnostic and predictive markers of antiphospholipid syndrome (APS). Clinical characteristics of APS; Laboratory diagnosis of APS; Factors influencing the long-term prognosis of APS; Common complications of APS.

Objective: To determine the prevalence of thrombocytopenia in a group of patients suffering from the antiphospholipid syndrome (APS) and to investigate whether these patients may have any particular clinical or serological features.Methods: Retrospective analysis. A group of 171 APS patients seen in our department were studied for the presence of thrombocytopenia. Clinical and serological features of these patients were analysed by standard methods and each of them was correlated to the presence of thrombocytopenia and compared with those found in the group without thrombocytopenia.Results: Each of the patients studied had a minimum of three platelet counts during the follow up period. Forty (23.4%) were found to have thrombocytopenia; 13 with persistently low and 27 patients with intermittently low platelet counts. There were no statistically significant differences in sex, age, disease duration or diagnosis when compared with the group of APS patients without thrombocytopenia. Thrombocytopenia was associated with thrombosis in 18, with miscarriages in five, and with both of these features in 13 patients. It was the only manifestation of the APS in four patients. All patients had persistently positive tests for antiphospholipid antibodies concomitantly with the low platelet counts. No significant association was found between the presence of thrombocytopenia and clinical or serological manifestations in APS patients.Conclusion: This study showed a prevalence of thrombocytopenia of 23.4% in APS. These patients did not present any significant clinical or serological features that distinguish them from those patients without thrombocytopenia. [ABSTRACT FROM AUTHOR]

Recurrent pregnancy loss is one of the main manifestations of antiphospholipid (Hughes) syndrome (APS). Fetal deaths (beyond the 10th week of gestation) are the most typical obstetric complication of APS. Data from controlled therapeutic trials are difficult to analyse, due to small samples and great heterogeneity in the obstetric history and antiphospholipid antibody profiles of women included. Corticosteroids are more harmful than beneficial. Heparin and aspirin are the drugs of choice for APS-related miscarriage, although it is not clear whether combination of both drugs is necessary for all women. The role of immunoglobulins is not well defined. [ABSTRACT FROM AUTHOR]

Hughes (antiphospholipid) syndrome is a condition in continuous evolution. Since it was first described almost 20 years ago, significant advances in its diagnosis and management have been made. Recently, classification criteria for definite antiphospholipid syndrome have been proposed and validated. However, there is still controversy regarding issues such as the utility of new diagnostic tests (detecting antibodies to β2-glycoprotein I, phospholipids other than cardiolipin or phospholipid mixtures), the role of antiphospholipid antibodies in neurological conditions such as demyelinating disease, cognitive impairment or migraine and the optimal management of thrombosis and obstetric manifestations. These are all briefly discussed in this review. [Copyright &y& Elsevier]