TMA 65 YEARS OLD TODAY

It was 65 years ago to day that Prof Symmers taught the world to say TMA.

Prof Symmers, or Professor William St Clair Symmers to give him his full name, was a Consultant in the General Hospital of the University of Birmingham (demolished in mid 1980s) in England.  His article in the British Medical Journal published on 25 October 1952 proposes the term “thrombotic microangiopathy” to describe a syndrome of diseases that were previously described as “TTP” among other names, including Moschowitz Disease, which had first been identified in 1924.

Symmers had searched through published cases in the 1940s and 1950s to identify 36 incidents of what he thought described thrombotic microangiopathy.  The rarity of the syndrome, as he saw it, was not because of incidence but due to a lack of “familiarity with the condition being an unusually important factor in its recognition”

An early case of “you must THINK TMA to diagnose it”!

30 of those cases had been recognised in the USA, and 3 in the UK. Of the USA 30, 17 were reported by three Centres in New York (Mount Sinai), Boston (Harvard) and Washington (Military).

Symmers’ article  ( to see click here )covered 31 of the 36 cases plus 2 new cases that he had a personal knowledge of. The patients ranged in age from 9.5 to 74 years but were mainly young adults and mostly women. The symptoms he described are still familiar today but were mainly haematological and neurological in nature and rarely renal.

All cases were fatal, most within 2 to 6 weeks of presentation. One patient survived for 3.5 years following a splenectomy. There were few treatment options, particularly few drugs, although Symmers mentions a steroid treatment, cortisone, in development. Blood transfusions only offered only transitory relief.

From the permitted post mortem studies, the capillaries in the myocardium and adrenal organs were most affected; but also the renal cortex where ” hyaline (transparent)  materials ,but not clots, obliterated the capillaries and appears to change the capillaries walls” which makes it surprising that renal failure symptoms did not feature more prominently.

The cause of TMA was unknown. The paper mentions idiopathic TTP and SLE (Lupus ), although there was much debate and dispute about a link. Similarly, another research suggestion that the TMA had resulted from bacterial, toxic or circulatory damage of the Endolethium was dismissed as unconvincing. It would be another three years before HUS gets its name.

The most important paragraph in the paper was the one headed “Nomenclature”. In it Symmers concludes none of  the then known named diseases adequately described what being found in  the syndromes he was studying; and so he offers thrombotic microangiopathic haemolytic anaemia as his alternative, or, for brevity thrombotic microangiopathy. He does not use, nor offer in his article , the abbreviation “TMA” for even greater brevity!

Symmers was clearly not seeing a spectrum of TMA* with overlaps between categories at that time. This is  something which has emerged in more recent times. But importantly the condition had been given  a name, and ,with that, the opportunity for it to be recognised  and understood.

This year is 100th anniversary of his birth; Prof. Symmers died at the age of 83 on this day 25 October in 2000.

*as an indication of how knowledge about TMA has moved on considerably since , consider this paper by some Newcastle upon Tyne UK researchers click here

PS.

Within it all is aHUS ,whether it is in the TMAs or the TTP categorisations.

Now the name aHUS has been going in and out of style and not guaranteed to remain a while. So, let’s introduce to you …PHUSS!  Click here

 

 

aHUS Alliance – Thrombotic Microangiopathy Symposium: Through the Lens of aHUS

 

ARTICLE 24 Aug 2017 Event, Multi-Disciplinary Approach (Med Ed Event, featuring 5 Boston area physicians)

VIDEO Playlist:  TMA Clinical Presentations.   Set of 9, each physician preceeded by aHUS patient experiences:  #TMAboston 24 Aug 2017