THROMBOTIC THROMBOCYTOPENIC PURPURA

Course overview

THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) IS A MICROANGIOPATHIC HEMOLYTIC ANEMIA CLASSICALLY CHARACTERIZED BY THE PENTAD OF FEVER, HEMOLYTIC ANEMIA, THROMBOCYTOPENIA, AND RENAL AND NEUROLOGIC DYSFUNCTION. TTP RESULTS FROM EITHER A CONGENITAL OR ACQUIRED ABSENCE/DECREASE OF THE VON WILLEBRAND FACTOR-CLEAVING PROTEASE ADAMTS13 (A DISINTEGRIN AND METALLOPROTEINASE WITH A THROMBOSPONDIN TYPE 1 MOTIF MEMBER 13). LOW LEVELS OF ADAMTS13 ACTIVITY RESULT IN MICROTHROMBI FORMATION, WHICH LEADS TO END-ORGAN ISCHEMIA AND DAMAGE. THE CENTRAL NERVOUS SYSTEM AND KIDNEYS ARE THE TWO MOST COMMON ORGAN SYSTEMS AFFECTED BY TTP. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF PATIENTS WITH THROMBOTIC THROMBOCYTOPENIC PURPURA AND HIGHLIGHTS THE ROLE OF INTERPROFESSIONAL TEAM MEMBERS IN COLLABORATING TO PROVIDE WELL-COORDINATED CARE AND ENHANCE OUTCOMES FOR AFFECTED PATIENTS.