do you have any information about the disease called ttp?

Question

has anyone known anyone with the disease of ttp?

Answer 1

TTP stands for thrombotic thrombocytopenic purpura. This is a life-threatening disorder affecting red blood cells, platelets (also known as thrombocytes), and the endothelial cells lining blood vessels. The key pathogenic feature of TTP is the formation of platelet aggregates within the microcirculation. The main clinical signs are thrombocytopenia, haemolytic anaemia, renal dysfunction, neurological signs, and fever. Modern treatments such as the infusion of freshly-frozen plasma can greatly reduce the risk of mortality from this disease. The underlying cause has been shown to be mutations in a gene that normally codes for an enzyme that acts on protein aggregates in the blood known as the von Willebrand factor.

Thrombotic thrombocytopenic purpura (TTP) is quite rare. It affects many systems of the body. The main feature of TTP is the formation of clumps of platelets (also known as thrombocytes) within the smaller blood vessels. (Platelets are formed in the bone marrow as tiny fragments of large cells called megakaryocytes, and play an important role in normal blood clotting.) In TTP there seems to be an abnormal interaction between the lining of blood vessels and the platelets which leads to the formation of tiny blood clots (thrombosis) and proliferation of the cells forming the blood vessel walls. Patches of purplish discolouration appear because of damage to the vessels and the blood within them, hence the inclusion of ‘purpura’ in the name. These changes occur with only minimal inflammation but they are serious because they result in hemolytic anaemia (the red blood cells disintegrate), neurological symptoms, renal disease, and fever. TTP can occur in any age group but is most often seen in patients 30 to 50 years of age. Several factors seem to predispose towards the development of TTP, including infections, drugs, menses, pregnancy, autoimmune diseases, cancer, and bone marrow transplantation.

Until recently, the primary cause of TTP was unknown. Research suggested that the damage to the lining of blood vessels was triggered by plasma factor(s) that lead to the release of an abnormal von Willebrand factor which then causes platelet aggregation (Eldor, 1998). There was evidence that the condition was an autoimmune disease in which the immune system turned against normal cells and molecules within the body. However, it was considered to be premature to classify TTP as an autoimmune disease (Porta, Caporali, and Montecucco, 1999). The most recent research has shown that mutations in a member of the ADAMTS gene family on chromosome 9 is probably the underlying genetic cause of TTP (Levy et al, 2001). The gene ADAMTS13 codes for an enzyme that has the normal function of cleaving the aggregates of von Willebrand factor proteins, and in this way modulating the delicate interrelationships between platelets and blood vessel linings.

TTP has been treated with plasma exchange since the 1960s, and the outlook now is much improved. A variety of supportive treatments have been tried in combination with plasma exchange, for example: corticosteroids, vincristine, aspirin, and dipyridamole (Brailey, Brecher, and Bandarenko, 1999; Creager, Brecher, and Bandarenko, 1998). Cure is possible, although approximately half the patients will relapse after a disease-free period. Removal of the spleen seems to be helpful for people with relapsing TTP (Veltman et al, 1995). The recent identification of the genetic cause of TTP by Levy et al (2001) has opened the possibilities of specific enzyme replacement therapy and gene therapy in the future.

Answer 2

hi ,

what does ttp mean ? u have to give the full form if u want an answer

Dr.Ashwini