Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic Thrombocytopenic Purpura (TTP) is a rare and potentially life-threatening blood disorder characterised by widespread formation of tiny clots within small blood vessels. These clots consume platelets and damage red blood cells, leading to thrombocytopenia and microangiopathic haemolytic anaemia. TTP most commonly results from severe deficiency of the ADAMTS13 enzyme, which normally regulates the size of von Willebrand factor. The deficiency may be inherited or, more commonly, acquired when the immune system produces antibodies against ADAMTS13.

Symptoms can develop suddenly and may include unusual bruising, pinpoint skin spots, fatigue, weakness, headache, confusion, fever, abdominal discomfort, or reduced urine output. Neurological and kidney complications can occur because small blood-vessel clots restrict blood flow to vital organs. Diagnosis involves a combination of blood tests, including a complete blood count, blood film examination, haemolysis markers, and ADAMTS13 activity testing.

TTP is a medical emergency requiring immediate treatment. Plasma exchange is the cornerstone of therapy and removes harmful antibodies while replacing functional ADAMTS13. Corticosteroids and other immune-directed treatments may also be used. Early recognition and treatment are essential because untreated TTP can rapidly cause severe organ damage or death.