Final answer:
Thrombotic thrombocytopenic purpura (TTP) is a serious disease caused by a deficiency in the ADAMTS13 enzyme, leading to widespread formation of small blood clots and is treated with plasma exchange and immunosuppression.
Step-by-step explanation:
The disease linked to a deficiency in ADAMTS13 is called thrombotic thrombocytopenic purpura (TTP). ADAMTS13 is an enzyme that is responsible for cleaving von Willebrand factor, preventing the formation of inappropriate blood clots. A deficiency in this enzyme can lead to the development of small blood clots throughout the body, which can be life-threatening and is considered a medical emergency. TTP is characterized by a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, renal dysfunction, and fever. TTP can be triggered by various factors, including infections, pregnancy, and certain medications, and requires prompt treatment, commonly with plasma exchange and immunosuppressive therapy.