TTP is characterized by a severe reduction in platelet count, microangiopathic hemolytic anemia (low hemoglobin, elevated bilirubin, elevated LDH), neurological manifestations such as confusion or seizures, renal involvement, and, in some cases, fever or gastrointestinal symptoms [2,10] Although these manifestations are largely nonspecific, they may precede acute complications, including respiratory distress, infections, and treatment refractoriness [1,13,14].In the absence of prompt testing of ADAMTS13 activity, rapid recognition of these signs becomes critical. The gene discussed is ADAMTS13; the disease is congenital thrombotic thrombocytopenic purpura.