thrombotic thrombocytopenic purpura (updated)

Image linked from mrbown

Sondra, aka la idler
RIP 30/11/05

It's never easy facing death and almost taboo to talk about it after it happens but facing up to unknowns and uncomfortable issues is one of the ways we overcome.

mrbrown has learnt that la idler died from an underlying condition that resulted in thrombotic thrombocytopenic purpura or TTP.

Just what does that long medical term means? Essentially it describes a syndrome and not the underlying disease. A syndrome is a host of signs and/or symptoms, when taken together sometimes points to an underlying cause, sometimes we don't know what is happening. And when the medical establishment doesn't know what is the underlying cause, the prefix idiopathic is added to the term. Hence if a medical reports states that the patient had idiopathic TTP or idiopathic high blood pressure, it simply means we don't know what's causing the TTP or high BP respectively.

Thrombotic comes from the word thrombus, the latter meaning blood clot. As some may know, blood platelets is one of the important component involved in the complex process of clotting. Another name for platelet is thrombocyte.

Thrombocytopenic means a decreased concentration of platelets, making formation of blood clots difficult and sometimes a person who is thrombocytopenic will experience unexplained bleeding. And why would a normal person bleed? Well, what is not widely known is that cells in our bodies breakdown regularly and repairs are an ongoing process. So the cells in a normal person walking around are being continually replaced, ensuring that he/she runs around without problems.

But didn't I just mentioned that thrombosis means clotting? So isn't thrombotic thrombocytopenic an oxymoron, given that a person with low platelet count has problems getting the blood to clot? Well, it seems that the initial unexplained thrombosis uses up so much platelets that there isn't enough to go around to repair other places that need to clot. Therefore, in TTP the patient's blood clots and bleeds at inappropriate places.

Purpura simply means bruising.

Taken together, TTP means random, uncontrolled clotting with low platelet counts and bruising.

A person with TTP usually presents with fever, headache, malaise (general sense of feeling unwell) and diarrhoea. Brusing and small bleeds also occurs in the gums and nose. Investigations would reveal a low platelet count. These are also the usual signs/symptoms in dengue patients and it is only by testing for the presence of anti-dengue antibodies can we confirm that the patient has contracted dengue or not. I suspect that the initial management for suspected TTP is similar for dengue hemorraghic fever. TTP is a rare disorder, with about 1 in 1-3 million cases annually although women seemed to be affected more than men. TTP may result from an inherited condition or it may be acquired. The actual causes are possibly many.

See this link for a better explanation.

Whatever the case may be. Rest in peace, Sondra.


Update
For those with access to scientific papers here are some useful references...

Yarranton H, Machin SJ. 2003. CURRENT OPINION IN NEUROLOGY 16:367-373

Richards A, Goodship JA, Goodship THJ. 2002. CURRENT OPINION IN NEPHROLOGY AND HYPERTENSION 11:431-435

Tsai HM, Lian ECY. 1998. NEW ENGLAND JOURNAL OF MEDICINE 339:1585-1594

Egerman RS, Witlin AG, Friedman SA, Sibai BM. 1996. AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY 175:950-956


Technorati tags: Thrombotic Thrombocytopenic Purpura, Medical, Blood Disorder