Thelansis

  Congenital thrombotic thrombocytopenic purpura (cTTP) is an inherited condition characterized by severe reduction of platelets in the bloodstream, destruction of red blood cells, and varying degrees of organ failure. While most individuals with this condition experience symptoms in infancy or childhood, the severity and specific manifestations can differ significantly. Some individuals may only have mild symptoms of low platelet count throughout childhood. In contrast, others may develop significant jaundice and experience episodes of low platelet count and red blood cell destruction soon after birth. In some cases, the initial onset of TTP occurs during adulthood, particularly in women, and is triggered by pregnancy. Infections and vaccinations can also act as precipitating factors. Congenital TTP is caused by a mutation in the ADAMTS13 gene located on chromosome 9q34. This gene provides instructions for producing ADAMTS13, an enzyme breaking down excessively large von Willebr...