Open Access<p>Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes</p>
Author(s) -
Selin Küçükyurt,
Ahmet Emre Eşkazan
Publication year - 2020
Publication title -
journal of blood medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.676
H-Index - 18
ISSN - 1179-2736
DOI - 10.2147/jbm.s205630
Subject(s) - medicine , adamts13 , microangiopathic hemolytic anemia , thrombotic microangiopathy , rituximab , thrombotic thrombocytopenic purpura , decompensation , asymptomatic , exacerbation , gastroenterology , refractory (planetary science) , intensive care medicine , disease , platelet , physics , lymphoma , astrobiology
Acquired or immune-mediated TTP (iTTP) is a life-threatening thrombotic microangiopathy, characterized by the presence of microangiopathic hemolytic anemia and severe thrombocytopenia, and a variable degree of ischemic organ damage, related to a severe deficiency of ADAMTS13, which is a serine metalloprotease necessary for cleavage of large vWF multimers. There has been a dramatic decrease in mortality rates with the recognition of the pathophysiology of iTTP over the years. Although therapeutic plasma exchange (TPE) together with corticosteroids are the backbone of the upfront treatment of patients with iTTP with successful outcomes, patients may remain refractory and/or relapse following an initial response to this treatment.