Intracranial hemorrhage in immune thrombotic thrombocytopenic purpura treated with caplacizumab

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening condition resulting from IgG autoantibodies to 'a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13' (ADAMTS13). Severe ADAMTS13 deficiency causes accumulation of ultra-large von Willebrand factor (VWF) multimers which bind platelets, leading to widespread microvascular thrombosis, compromise of critical organs and profound thrombocytopenia1,2 . In the absence of treatment, TTP is usually fatal.

[1]  É. Azoulay,et al.  A regimen with caplacizumab, immunosuppression and plasma exchange prevents unfavorable outcomes in immune-mediated TTP. , 2020, Blood.

[2]  S. Lane,et al.  Real-World Evidence of Caplacizumab Use in the Management of Acute TTP. , 2020, Blood.

[3]  S. Vesely,et al.  ISTH guidelines for treatment of thrombotic thrombocytopenic purpura , 2020, Journal of thrombosis and haemostasis : JTH.

[4]  A. Kribben,et al.  Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura. , 2020, Blood advances.

[5]  W. Dzik,et al.  Deaths and complications associated with the management of acute immune thrombotic thrombocytopenic purpura , 2020, Transfusion.

[6]  A. E. Eşkazan,et al.  Caplacizumab as an emerging treatment option for acquired thrombotic thrombocytopenic purpura , 2019, Drug design, development and therapy.

[7]  Flora Peyvandi,et al.  Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura , 2019, The New England journal of medicine.

[8]  S. Vesely,et al.  Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. , 2017, Blood advances.

[9]  B. Jilma,et al.  Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura. , 2016, The New England journal of medicine.

[10]  Ang Li,et al.  Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative , 2015, British journal of haematology.

[11]  M. Scully,et al.  Rituximab for thrombotic thrombocytopenic purpura: benefit of early administration during acute episodes and use of prophylaxis to prevent relapse , 2013, Journal of thrombosis and haemostasis : JTH.

[12]  H. Deckmyn,et al.  Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon (Papio ursinus). , 2010, Blood.

[13]  J. Cavenagh,et al.  Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS‐13 , 2007, British journal of haematology.

[14]  G. Hutchins,et al.  Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. , 2003, Archives of pathology & laboratory medicine.