Can thrombotic thrombocytopenic purpura be cured? What are the methods?

Thrombotic thrombocytopenic purpura is a disease that most people have never heard of. Many people don’t know what treatment method to choose. Especially some patients with this disease always feel that they have some difficult and complicated diseases. They do not receive treatment for a long time and become depressed. In fact, as long as everyone can discover this disease in time, effectively control and treat it, and choose the treatment method suitable for their condition, they can not only get rid of the pain of the disease, but also have the hope of cure.

1. Plasma exchange therapy is the preferred treatment method. Since 1976, the efficacy of this method in treating TTP has increased rapidly, reaching 67% to 84%, greatly improving the prognosis of TTP. It is believed that it can remove platelet-promoting aggregation substances in the body and replenish normal anti-aggregation substances, and should be performed as early as possible. The general dosage is 40 to 80 ml/kg of fresh frozen plasma per day for at least 5 to 7 days. If the treatment is effective (usually within 1 to 2 weeks), the serum LDH concentration will decrease, the platelet count will increase, and the nervous system symptoms will recover. Usually, plasma exchange can be stopped when the serum LDH concentration drops to 400U/L. It is not advisable to use cold precipitate in plasma exchange therapy to avoid large amounts of factor ⅤW triggering platelet aggregation in blood vessels. Platelet transfusion should be listed as a contraindication.

2. Adrenal cortical hormones are less effective in treating TTP when used alone. Generally, prednisone is started at 60 to 80 mg/d and increased to 100 to 200 mg/d if necessary. Those who cannot take the medicine orally can also use corresponding doses of hydrocortisone or dexamethasone. There is no consensus on the treatment of acute primary TTP, and hormones may aggravate the formation of platelet thrombi. Some people also believe that the effectiveness of hormones alone is only 11%, and it should be used in combination with other methods to achieve higher efficacy.

3. Vincristine (VCR) is often used as an immunosuppressant, especially for those with elevated PAIgG. Schreeder et al. (1983) treated two cases of TTP with vincristine (VCR) and achieved remission in both cases. The first case achieved partial remission after plasma exchange therapy and splenectomy with temporary effect, but achieved complete remission after vincristine (VCR) treatment. Joel et al. (1985) reported a case of chronic TTP complicated by factor ⅤW deficiency in a 57-year-old woman. After treatment with prednisone, azathioprine and plasma exchange, TTP was relieved and factor ⅤW disappeared after 21 days. In recent years, some people have reported that vinblastine (VCR) should be considered as the initial treatment for this disease, with an effective rate of about 70%. Pallavicini (1994) proposed that vinblastine (VCR) is a promising drug for patients with TTP who are ineffective in plasma exchange and conventional drug treatment. Intravenous injection of 2 mg per week, 24 to 50 days after the start of treatment, a total amount of 6 to 14 mg of vincristine (VCR) can achieve complete remission. Its mechanism of action is: ① changing platelet glycoprotein receptors to prevent contact with factor VI and reduce platelet aggregation; ② playing an immunomodulatory and immunosuppressive role on the endothelial cells of the blood vessel wall. Fulminant or progressive cases can be treated with drugs such as mitomycin, cyclosporine, and cisplatin.

4. Supplement ADAMTS13 protein plasma to purify ADAMTS13 protein . Cloning the ADAMTS13 gene and obtaining functional ADAMTS13 recombinant protein are still in the experimental research stage and are currently the most promising treatment for TTP. Theoretically, the use of rh-ADAMTSl3 as a replacement therapy for patients with hereditary TTP would be a promising treatment option.