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
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References
Caplacizumab Package Insert: accessed 6/26/22 - https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/761112s000lbl.pdf.
- Caplacizumab for acquired thrombotic thrombocytopenic purpura.NEJM. 2016; 374: 511-522
- Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura.NEJM. 2019; 380: 335-346
- Derivation and external validation of the PLASMIC Score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.Lancet Haematol. 2017; 4: e157-e164
- Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.Haematologica. 2012; 97: 1181-1186
- ISTH guidelines for treatment of thrombotic thrombocytopenic purpura.J Thromb Haemost. 2020; 18: 2496-2502
- Real-world experience with caplacizumab in the management of TTP.Blood. 2021; 137: 1731-1740
- Successful management of three patients with autoimmune thrombotic thrombocytopenic purpura with paradigm-changing therapy: caplacizumab, steroids, plasma exchange, rituximab, and intravenous immunoglobulins (CASPERI). Transfusion Apheresis.Sciences. 2021; 60103011
- Cost effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura.Blood. 2021; 137: 969-976
- ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP.Blood. 2020; 4: 3093-3103
Article info
Publication history
Published online: February 10, 2023
Accepted:
February 9,
2023
Received in revised form:
February 3,
2023
Received:
January 22,
2023
Publication stage
In Press Journal Pre-ProofFootnotes
☆This article was completed without external funding.
Identification
Copyright
© 2023 Elsevier Ltd. All rights reserved.
