Caplacizumab as rescue therapy in refractory TTP involving neurologic features

Transfus Apher Sci. 2023 Jun;62(3):103656. doi: 10.1016/j.transci.2023.103656. Epub 2023 Feb 11.

Abstract

Background: The role of caplacizumab in the routine treatment of immune thrombotic thrombocytopenic purpura (iTTP) remains to be established.

Case summary: A 56-year-old woman was transferred to our center with iTTP and neurologic features. At the outside hospital, she was initially diagnosed and managed as Immune Thrombocytopenia (ITP). Upon transfer to our center, daily plasma exchange, steroids, and rituximab were initiated. After an initial improvement, refractoriness became evident with a decline in platelet count and continued neurologic abnormalities. Initiation of caplacizumab resulted in rapid hematologic and clinical responses.

Conclusion: Caplacizumab is a valuable treatment modality in iTTP, particularly in cases associated with refractoriness or neurologic features.

Keywords: ADAMTS13; Caplacizumab; Plasma exchange; Rituximab; TTP.

Publication types

  • Case Reports

MeSH terms

  • ADAMTS13 Protein
  • Female
  • Humans
  • Middle Aged
  • Plasma Exchange
  • Platelet Count
  • Purpura, Thrombocytopenic, Idiopathic* / drug therapy
  • Purpura, Thrombotic Thrombocytopenic* / drug therapy
  • Rituximab / therapeutic use
  • Single-Domain Antibodies* / therapeutic use

Substances

  • caplacizumab
  • Rituximab
  • Single-Domain Antibodies
  • ADAMTS13 Protein