The blood picture of TTP is rather characteristic. Massive hemolysis leads to polychromasia and the early release of normoblasts. Schistocytes indicate microangiopathy. In addition, thrombocytopenia is also present.Abstract:
Synonym = Moschcowitz syndrome. TTP is a clinical syndrome with microangiopathic hemolytic anemia (MAHA), thrombocytopenia and changing neurologic findings. Fever and kidney dysfunction occur frequently. TTP bears a striking resemblance to the hemolytic-uremic syndrome (HUS) of childhood. Abnormalities in von Willebrand factor processing lead to platelet thrombosis and microangiopathic red cell destruction.
Clinical picture:
TTP is rare. It can occur at any age. A distinction is made between the chronic, recurrent form and the acute fulminant syndrome. The latter has a high mortality rate. This can be markedly lowered by early recognition of the syndrome and immediate initiation of therapy (plasmapheresis with fresh-frozen plasma). Symptoms are varied due to the occurrence of microthrombi in various organs.
Hematology:
The blood picture of TTP is rather characteristic. Massive hemolysis leads to polychromasia and the early release of normoblasts. Schistocytes indicate microangiopathy. In addition, thrombocytopenia is also present.