Recently, two of my heroes died. Gerry Weissmann and Chuck Christian.
Gerry was a giant in American medicine, Dean of NYU (New York University) and internationally renowned as a polymath – an international expert on a wide variety of topics. I knew him well and was a huge admirer. Once, years ago, we were both speaking at an international rheumatology meeting in Rio. One evening we were guests at a speakers’ dinner, at a restaurant in the hills above Rio. Very exclusive with a bongo band playing.
Suddenly, there were several ‘bang bangs’ – gunshots. The bongo band dived under two tables. The rheumatology guest stayed put – amateurs! After a few minutes, the owner of the restaurant come in and apologised. “I’m sorry – we had a precautionary shooting!”
The other admired colleague in Prof Charles Christian – my boss during my two years in New York and my hero. The hospital for special surgery in New York is holding a memorial service in New York in October and I am planning to be there, even if it’s a 24-hour round trip on Norwegian Air! I’ll keep you posted in November!
Patient of the month : Blood tests
Mrs LL aged 51, like a number of our patients, had been diagnosed with Lupus some 30 years before. Clinically the Lupus was mild and mainly responsive to Plaquenil alone. But interestingly, her tests showed up a positive test for syphylis (a positive “W.R.”). [Medical students will know that the old test of syphilis – the ‘WR’ (Wasserman Reaction) could give ‘false positive’ results in some individuals – notably, we now know, in some individuals with Hughes Syndrome].
At the age of 20, she married. But, the dream of pregnancy became a nightmare. Firstly, she took more than two years to conceive, only to suffer a miscarriage.
Followed by another – and five more.
And to add to her very sad story, the seventh pregnancy ended in a late pregnancy loss – a stillbirth – to quote a leader in The Times, “one of the greatest tragedies known to mankind”.
Mrs LL and her husband decided on no more pregnancies. Mrs LL remained well for several years, the Lupus in remission. Then, following a long car journey to Scotland, she developed a leg DVT (deep vein thrombosis).
She was given the conventional 6 months’ Warfarin. However, noting her obstetric history, the registrar asked for test for ‘aPL’ – antiphospholipid antibodies.
The results came back positive: of the 3 conventional tests, 2 were positive –
Anticardiolipin (ACL) ‘strongly positive’
Lupus anticoagulant (LA) ‘positive’
Anti-Beta-2 GP1 ‘negative’
In summary, a healthy young woman with a terrible obstetric history, a recent DVT, and 2 out of 3 APL tests positive.
How would you treat that?
One of my concerns over the last 35 years since our description of the anti-phospholipid syndrome, has been the follow-up of young women with ‘obstetric APS’. A successful pregnancy on heparin and aspirin, say, but what of the future? A DVT? A stroke? Ongoing neurological problems including memory loss? Is there any way of forecasting the risk? To be fair, the combined experience of those working in this field have come out with some broad lessons. Will they hold true?
1. Venous thrombosis, e.g. DVT, may not require as aggressive anticoagulation as arterial.
2. A vein clot in which there may be a triggering event (e.g. a long car journey?) may also require a less aggressive approach.
3. Recent studies have suggested that to have all three APL tests positive (“triple positive”) forecasts a poorer outcome, as far as thrombosis risk is concerned.
How would Mrs LL future treatment hold out? At present, venous clot versus arterial; less aggressive maybe?
Only 2 of the 3 currently used tests positive? Statistics are one thing. Clinical practice is another.
I ask myself the question, who wants a stroke? Probably the lesser evil is the “take the medicine” – aspirin daily, or even perhaps one of the newer oral anticoagulants. But should ‘brain’ symptoms such as severe migraines or T.I.A.’s creep in, think Warfarin.
One last point. This young lady had a background history of Lupus. Although, clinically, the Lupus was in remission and the tests negative, another possible treatment choice might be to go back to Plaquenil (1 tablet of 200 mg a day).
As well as being a safe and effective treatment in Lupus, it also has some anti-clotting properties – hence is occasional use in Hughes Syndrome pregnancies.