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Pathology 2018
Research & Reviews: Journal of Medical and Health Sciences
ISSN: 2319-9865
Page 74
October 08-09, 2018
Edinburgh, Scotland
17
th
International Conference on
Pathology & Cancer
Epidemiology
T
hrombotic thrombocytopenic purpura (TTP) is one of
the most serious and life-threatening form of thrombotic
microangiopathy characterized by microangiopathic hemolytic
anemia, severe thrombocytopenia, and multiple organs damage
due to VWF–platelet aggregations in the arterioles and capillaries
of end organs. The thrombi are found most extensively in the
heart, brain, kidney, pancreas, spleen, mesentery and adrenal
glands. Thrombocytopenia results from consumption of platelets
in the thrombotic process, while erythrocyte fragmentation and
hemolysis result from mechanical injury induced by abnormally
high shear stress in the microvasculature. Pathophysiology
involves the absence of von Willebrand factor cleaving enzyme
(ADAMTS-13), resulting in unusually large von Willebrand
multimers. These multimers lead to platelet aggregations,
microthrombi and subsequent thrombocytopenia. About 35% of
adult patients have idiopathic (acquired) TTP due to formation
of antibodies/inhibitors against ADAMTS13 (a disintegrin and
metalloprotease with thrombospondin type 1 repeats, member
13). These patients develop severe ADAMTS13 deficiency and
acute TTP. The inhibitors are primarily IgG but less often IgM.
The plasma ADAMTS13 activity level is less than 10% or 5% in
acute TTP. Inherited form of TTP is also described in children
due to mutations of ADAMTS13 gene located on the long arm of
chromosome 9 An ADAMTS13 level >10% excludes the diagnosis
of TTP. Early diagnosis of TTP is essential to initiate appropriate
treatment. The first-line therapy for acute TTP is based on daily
therapeutic plasma exchange supplying deficient ADAMTS13.
Immune modulators are humanized anti-CD20 monoclonal
antibody rituximab.
qaiserhasnain123@yahoo.comThrombotic thrombocytopenic purpura and the role of
ADAMTS13 as a key enzyme in the pathogenesis of TTP
Qaiser Hasnain
Amna Inayat Medical College - University of Health Sciences, Pakistan
RRJMHS 2018
Volume: 7