The therapy of an acute TTP episode has to be started as early as possible. The standard treatment is the daily replacement of the missing ADAMTS13 protease in form of plasma infusions or in more severe episodes by plasma exchange. In the latter the patients plasma is replaced by donated plasma. The most common sources of ADAMTS13 is platelet-poor fresh frozen plasma (FFP) or solvent-detergent plasma. The benefit of plasma exchange compared to plasma infusions alone may result from the additional removal of ULVWF. In general both plasma therapies are well tolerated, several mostly minor complications may be observed. The number of infusion/exchange sessions needed to overcome a TTP episode are variable but usually take less than a week in USS. The intensive plasma-therapy is generally stopped when platelet count increases to normal levels and is stable over several days. Preventive therapy Not all affected patients seem to need a regular preventive plasma infusion therapy, especially as some reach long-term remission without it. Regular plasma infusions are necessary in patients with frequent relapses and in general situations with increased risk to develop an acute episode (as seen above) such as pregnancy. Plasma infusions are given usually every 2–3 weeks to prevent acute episodes of USS, but are often individually adapted.
Upshaw–Schulman syndrome
Haglund's syndrome
Potocki–Shaffer syndrome
Frey's syndrome
Perlman syndrome
Urethral syndrome
Lamb-Shaffer syndrome
Shone's syndrome