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Medicine Bleeding and Thrombotic Disorders 0b342988

ADAMTS deficiency is seen in?

A
Essential thrombocythemia
B
ITP
C
Thrombotic thrombocytopenic purpura
D
CLL
High-Yield Explanation
Ans. is 'c' i.e., Thrombotic thrombocytopenic purpurao Patients with TTP are deficient in an enzyme called ADAMTSB. (This enzyme is also known as vWf metalloprotease.)o This enzyme normally degrades very high molecular weight multimers of von-Willebrandfactor. In the absence of this enzyme, these multimers of vWF accumulate in plasma and under some circumstances promote platelet macroaggregate formation throughout the microcirculation leading to symptoms of TTP.Thrombotic Thrombocytopenic PurouraAbsent AD AMTS|Accumulation of multimers of vWF|Platelet microaggregate formation throughout the microcirculation|T.T.P.

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