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. 2023 Jun 30:8:11.
doi: 10.21037/aob-21-87. Epub 2022 Apr 1.

How should caplacizumab be used for treatment of immune thrombotic thrombocytopenic purpura?

Lucy Zheng  1 , X Long Zheng  1
Affiliations

How should caplacizumab be used for treatment of immune thrombotic thrombocytopenic purpura?

Lucy Zheng et al. Ann Blood. .
No abstract available

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Conflict of interest statement

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://aob.amegroups.com/article/view/10.21037/aob-21-87/coif). XLZ serves as an unpaid editorial board member of Annals of Blood from March 2022 to February 2024. XLZ served as a speaker for Alexion and Sanofi, which terminated since last year. He is serving as a consultant for several companies including Alexion, Sanofi, Takeda, and Biomedica. He is also the co-founder of Clotsolution. He received honorarium from several universities and professional conferences as a keynote speaker. The other author has no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Schematic diagram demonstrates the process for the diagnosis and management of iTTP. When a patient presents with severe thrombocytopenia with platelet count <30×109/L, creatine <2.26 mg/dL in the presence of hemolysis and schistocytes, but no history of malignancy and/or hematopoietic progenitor cell transplantation or known drugs that causes TMA, the likelihood of iTTP is very high (based on French Score). This patient will be benefited from ordering an ADAMTS13 test, immediate TPE and corticosteroids, and early caplacizumab. Otherwise, if the clinical probability of iTTP is low to intermediate, ADAMTS13 test should be ordered prior to TPE and corticosteroid, but no caplacizumab. Use ADAMTS13 activity result to guide further therapies as shown in the bottom of the diagram. This figure is adapted from Zheng et al. (15). *, in most initial iTTP cases, plasma ADAMTS13 activity is less than 5 U/dL (or 5% of normal) or undetectable. Normal ADATMS13 activity is 50–150 U/dL. Those with a relapsed episode may have higher ADAMTS13 activity. TMA, thrombotic microangiopathy; iTTP, immune thrombotic thrombocytopenic purpura. TPE, therapeutic plasma exchange.
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