In ‘lockdown’ now for 2 months.  Almost a way of life.  The only redeeming factor has been the wonderful weather here in the South East of England. Cloudless blue skies day after day.

For people stuck in a block of flats, the past 2 months must have been so, so difficult.  One can only hope that, perhaps in a month or two, the strength of the corona plague will become less ferocious.

Patient of the Month

Ms V.S., a 55 year old PA to a manager of a clothing company, had a long and troublesome history.

She suffered from a jerking movement, affecting her arm and her head.  It was variable in its intensity but was definitely made worse by stress.

In a consultation, or an interview situation, Ms V.S’s affliction dramatically worsened, leading some of her friends to suggest that the problem was, at least, partly emotional.

But Ms V.S., on investigations, was found to have another medical problem.  Her blood tests showed a low platelet count – averaging 40,000 – 50,000 instead of the normal 150,000,  The count varied, often up to 100,000 but sometimes dipping to 20,000 or less.  Over the years, the treatment had been conservative, occasional severe ‘dips’ being treated with a course of steroids.

The rest of Ms V.S’s history was essentially negative, with one important caveat.  She had been attending a clinic with a history of severe headaches, often migrainous.

Investigations revealed positive aPL antibody tests – high levels of anticardiolipin antibodies.

Her initial treatment was with a course of low dose aspirin (75mg daily) – ‘trial and error’.  The result was striking.  The headaches improved, and the platelet count stabilised (though remaining lowish).

What is the patient teaching us?

Following our publications in 1983, describing the antiphospholipid syndrome, our team embarked on a series of collaborative studies with experts on liver, skin, neurology and cardiology.  We linked up with haematology, testing numbers of patients with platelet problems, and found that a significant number of patients with ‘idiopathic’ (cause not known) low platelet counts had positive aPL tests.

Could it be that the clotting tendency in Hughes Syndrome was due to abnormalities in platelet function?  The answer was ‘yes’ – in part.

Thanks to the work of Dr Munther Khamashta, who joined my unit from Spain in 1986, it became clear that abnormal platelet function (sometimes mild, sometimes severe) was common in Hughes Syndrome – including Ms V.S.  It is probably one of a number of causes of the abnormal clotting process in the syndrome

Footnote: 

Ms V.S’s two problems were neurological (tics and movement disorders) and haematological (abnormal platelet function).

It is notable that over the past several decades and more, these two clinical features have been linked.  Clearly an incentive for more research.

Professor Graham R V Hughes MD FRCP

London Lupus Centre

London Bridge Hospital