"Unveiling Thrombotic and Thrombocytopenic Purpura: Pathogenesis, Clinical Features, and Treatment Strategies"
Thrombotic and Thrombocytopenic Purpura Definition: TTP occurs in familial or acquired forms. Pathogenesis: Deficiency of ADAMTS13 metalloprotease leads to ULVWF multimers. Causes: Familial forms involve ADAMTS13 mutations; Acquired forms result from inhibitory IgG autoantibodies. Clinical Features: Pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal failure, fever. Treatment: Plasma exchange with FFP or cry supernatant, rituximab, alacizumab, and immunosuppressive therapies. TTP is type of Thrombocytopenia mean platelets count decrease its normal count. TTP is rare blood disorder characterized by clotting in small blood vessels (Thromboses) but unnecessary clot resulting in a low platelet count . Normally clotting present in tissue & vessels injury . In TTP, Patient have defect in (ADAMTS13) enzyme. ( For clearing the VWF multimers) Normally Gene to code for different protein. ADAMTS13