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Thrombotic thrombocytopenic purpura
A rare disease characterised by:
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Low platelet count
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Microangiopathic haemolytic anaemia (breakdown of red blood cells
through factors in the small blood vessels)
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Fluctuating neurological symptoms, such as bizarre behaviour or
headaches
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Renal function loss
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Fever
Causes
The disease is caused by spontaneous aggregation of platelets and
activation of coagulation in the small blood vessels. Recently, the
reason for this phenomenon has been identified to be the inhibition of
an enzyme by the name of ADAMTS13 by antibodies; it is therefore an
autoimmune disease. ADAMTS13 breaks down the so-called von Willebrand
factor (vWF), a protein that links platelets, blood clots and the blood
vessel wall in the process of blood coagulation. Very large vWF
molecules are more prone to lead to coagulation.
A hereditary form of TTP is called the Shulman-Upshaw syndrome. Other
rare causes are certain medications
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Symptoms and signs
It is very hard to disentangle TTP from Hemolytic Uremic Syndrome (HUS).
Both have very similar clinical characteristics, although the renal
symptoms tend to be more outspoken in HUS. HUS is often preceded by a
case of bloody diarrhea caused by the E. coli O157:H7 bacterium.
Treatment
TTP is generally treated by repeated plasmapheresis, which is the
removal of the patient's blood plasma and replacement with donor's
plasma.
Prognosis
Prognosis is reasonable: 90% survive an attack of TTP.
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