This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Bleeding and Thrombotic Disorders, Subject name: Medicine
ADAMTS deficiency is seen in? 
Select and explain. 
A. Essential thrombocythemia, B. ITP, C. Thrombotic thrombocytopenic purpura, D. CLL
C: Thrombotic thrombocytopenic purpura
Answer: C: Thrombotic thrombocytopenic purpura 
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.