Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially life-threatening blood disorder characterized by the formation of small blood clots throughout the body. The condition is characterized by two main features, namely thrombocytopenia (low platelet count) and microangiopathic hemolytic anemia (destruction of red blood cells).
In TTP, the underlying cause is often a deficiency in a specific enzyme called ADAMTS13, which is responsible for breaking down a protein called von Willebrand factor. When the levels of ADAMTS13 are low, von Willebrand factor is not properly broken down, leading to the formation of blood clots in small blood vessels.
These blood clots can obstruct normal blood flow and cause damage to organs such as the brain, heart, kidneys, and intestines. Common symptoms include widespread bruising (purpura), fatigue, weakness, fever, neurological abnormalities, and signs of organ dysfunction.
The diagnosis of TTP is made based on clinical symptoms, blood tests to determine platelet count and hemolytic anemia, and further testing to measure the levels of ADAMTS13 activity. Treatment typically involves plasma exchange or plasma infusion to replace the missing enzyme and remove the abnormal protein, along with immunosuppressive medications to suppress the immune response.
Early diagnosis and prompt treatment are crucial in TTP, as the condition can rapidly progress and lead to serious complications such as stroke or kidney failure. With appropriate treatment, the prognosis for TTP has improved significantly in recent years, although the condition may still have long-term effects on some individuals.
