Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes

Kucukyurt S., EŞKAZAN A. E.

JOURNAL OF BLOOD MEDICINE, cilt.11, ss.319-326, 2020 (ESCI) identifier identifier identifier

  • Yayın Türü: Makale / Derleme
  • Cilt numarası: 11
  • Basım Tarihi: 2020
  • Doi Numarası: 10.2147/jbm.s205630
  • Dergi Adı: JOURNAL OF BLOOD MEDICINE
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, EMBASE, Directory of Open Access Journals
  • Sayfa Sayıları: ss.319-326
  • İstanbul Üniversitesi Adresli: Hayır

Özet

Background: 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.