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Pathology DNB 2018 08d2c3e4

ADAMTS 13 is associated with which of the following disorders?

A
TTP
B
Churg Strauss syndrome
C
Wegener's granulomatosis
D
Membranous nephropathy
High-Yield Explanation
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

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