Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic Thrombocytopenic Purpura (TTP) is a rare but potentially fatal blood disorder characterised by the formation of tiny blood clots within small blood vessels throughout the body. These clots consume platelets, leading to thrombocytopenia (low platelet count), while also damaging red blood cells, causing microangiopathic haemolytic anaemia. The condition is most commonly associated with a severe deficiency of the ADAMTS13 enzyme, either inherited or caused by autoimmune antibodies that inhibit its activity.

Patients with TTP may experience unexplained bruising, fatigue, pale skin, fever, confusion, headaches, kidney dysfunction, or neurological symptoms such as seizures and stroke-like episodes. Because the disease progresses rapidly, early diagnosis and immediate treatment are essential to prevent life-threatening complications.

Diagnosis involves blood tests showing low platelets, fragmented red blood cells (schistocytes), elevated lactate dehydrogenase (LDH), and reduced ADAMTS13 activity. Prompt treatment usually includes plasma exchange therapy, corticosteroids, immunosuppressive medications such as rituximab, and newer targeted therapies like caplacizumab in appropriate cases. Supportive care may also be required to manage complications.

With timely treatment, survival rates have improved significantly. However, untreated TTP carries a very high mortality rate. Regular follow-up is important to monitor recovery, detect relapses, and manage long-term health risks associated with this serious haematological disorder.