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What is ADAM TSB.13 deficiency is seen in and explain in detail?
Answer- A. TTPTTP is included among group of diseases called thrombotic microangiopathic which are characterised by widespread thrombosis in microcirculation. The other disorder which is included in this group include hemolytic uremic syndrome (HUS)Small vessels like terminal aerioles and capillaries are involved.Pathogenesis of TTPPatients with TTP are deficient in an enzyme called ADAMTSB.T3 also known as vWF metalloprotease that degrades very high molecular weight multitimers of vWF.