

There are two main clinical features - clotting (thrombosis) and, in women, recurrent miscarriage. In the blood, the defining test is the presence of antiphospholipid antibodies (aPL).
There are enormous variations in presentation and in severity of Hughes' Syndrome, most, though not all, being explained on a basis of blood clotting. It is important to recognise that in this syndrome, as distinct from other (usually less common) clotting disorders, the thrombosis may happen both in the veins and in the arteries - the latter with much more severe consequences. The major features of the syndrome are listed in the table below.
1. Vein thrombosis |
• Deep vein thrombosis (DVT) e.g. arm or leg. |
2 Artery thrombosis |
• Brain - headaches, weakness, slurred speech ("transient |
3. Pregnancy loss |
• Early in pregnancy (miscarriage) or late ("foetal death") |
4. Low platelet count |
• Bruising (5-20%) |
For obvious reasons, the majority of cases currently seen by us have been diagnosed either because they have had a sudden clot, e.g., a leg thrombosis ("DVT") or recurrent clots, or else because they have been referred by an obstetrician who has made the diagnosis by aPL testing in a woman who has had a number of miscarriages.
In some, the history may be sudden, with the development of features suggestive of an early stroke or "transient ischaemic attack".
In others, the history may be longer, with, for example, headaches, or memory loss going back over a number of years.
The disease affects all age groups, from infancy (see later) to old age. However, the majority of patients are aged between 15 and 50. Females seem to outnumber males, though not to any great extent. These observations on age and sex may be flawed in that "referral patterns" may influence the figures. For example, because of the known association with pregnancy loss, there may well be a selection bias from pregnancy clinics.