Due to the high mortality of untreated<unk> , a presumptive diagnosis of<unk>  is made even when only microangiopathic hemolytic anemia and thrombocytopenia are seen, and therapy is started. Transfusion is contraindicated in<unk> <unk> , as it fuels the coagulopathy. Since the early 1990s, plasmapheresis has become the treatment of choice for<unk> . This is an exchange transfusion involving removal of the person's blood plasma through apheresis and replacement with donor plasma (fresh frozen plasma or cryosupernatant); the procedure must be repeated daily to eliminate the inhibitor and abate the symptoms. If apheresis is not available, fresh frozen plasma can be infused, but the volume that can be given safely is limited due to the danger of fluid overload. Plasma infusion alone is not as beneficial as plasma exchange. Corticosteroids (prednisone or prednisolone) are usually given. Rituximab, a monoclonal antibody aimed at the CD20 molecule on B lymphocytes, may be used on diagnosis; this is thought to kill the B cells and thereby reduce the production of the inhibitor. A stronger recommendation for rituximab exists where<unk>  does not respond to corticosteroids and plasmapheresis. Caplacizumab is an alternative option in treating<unk>  as it has been shown that it induces a faster disease resolution compared with those people who were on placebo. However, the use of caplacizumab was associated with increase bleeding tendencies in the studied subjects. People with refractory or relapsing<unk>  may receive additional immunosuppressive therapy, e.g. vincristine, cyclophosphamide, cyclosporine A, or splenectomy. Children with Upshaw-Schülman syndrome receive prophylactic plasma every two to three weeks; this maintains adequate levels of functioning ADAMTS13. Some tolerate longer intervals between plasma infusions. Additional plasma infusions may be necessary for triggering events, such as surgery; alternatively, the platelet count may be monitored closely around these events with plasma being administered if the count drops. Measurements of blood levels of lactate dehydrogenase, platelets, and schistocytes are used to monitor disease progression or remission. ADAMTS13 activity and inhibitor levels may be measured during follow-up, but in those without symptoms the use of rituximab is not recommended.
Thrombotic thrombocytopenic purpura
Atypical hemolytic uremic syndrome
Drug-induced thrombocytopenic purpura
Hemolytic–uremic syndrome
Immune thrombocytopenic purpura
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Gray platelet syndrome