STM Journals Peer-reviewed research from Consortium e-Learning Network

Research and Reviews: Journal of Oncology and Hematology

Thrombotic thrombocytopenic purpura: A Narrative Review of Diagnosis and Treatment by drugs and novel agents.

  1. Asad Jamal Ansari
  2. Shweta S
  3. Mohammad Monawwar
  4. Nishat Fatima

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an infrequent but fatal disease, is primarily caused by autoantibodies against the von Willebrand factor (VWF) cleaving metalloprotease ADAMTS13. Overall, severe deficiency of plasma ADAMTS13 action (< 10 IU dL-1) with or without noticeable inhibitory autoantibodies counter to ADAMTS13 supports the diagnosis of TTP. The International Working Group for TTP released a consensus statement on the standardization of terminology in these disorders which we use in this publication. Plasma exchange and corticosteroids remain to be the support of treatment, though, repurposed drugs and novel agents are evolving as effectual treatment options. In this review, new therapeutic expansions in immune mediated thrombotic thrombocytopenic purpura (TTP) counting bortezomib, N-acetylcysteine, caplacizumab, rituximab, and recombinant ADAMTS13, amongst others, are abridged.

Keywords

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