ADAMTS 13 is associated with which of the following disorders?: (A) TTP, (B) Churg Strauss syndrome, (C) Wegener's granulomatosis, (D) Membranous nephropathy
Answer is A. ADAMTS 13:- -Metalloprotease enzyme which inhibits VWF clumps. -cleaves VWF multimers into smaller monomers for eventual degradation Thrombotic Thrombocytopenic purpura (TTP)- Causes- Deficiency of ADAMTS 13 Ab formation against ADAMTS 13 - |ADAMTS 13 activity - VWF clumping - Platelet-Thrombi present C/F - Thrombocytopenia Microangiopathic Hemolytic anaemia Fever Renal failure CNS features Rx- Plasmapheresis