Should Caplivi (Ticlopidine) be discontinued based on ADAMTS13 (A Disintegrin and Metalloprotease with a Thrombospondin type 1 motif, member 13) levels?

From the Guidelines

Caplacizumab (Cablivi) should be discontinued when the ADAMTS13 activity level has normalized, as indicated by the most recent guidelines for the management of lupus nephritis and thrombotic microangiopathy (TMA) 1. The management of patients with lupus nephritis and TMA involves testing for ADAMTS13 activity and antibodies to ADAMTS13, as well as antiphospholipid antibodies, to determine the underlying etiology of TMA.

• According to the KDIGO 2024 clinical practice guideline for the management of lupus nephritis, patients with low ADAMTS13 activity (<10%) should be treated with plasma exchange and glucocorticoid, and may also receive caplacizumab, rituximab, and/or eculizumab 1.
• The guideline also recommends evaluating for thrombotic microangiopathy etiologies, including thrombotic thrombocytopenic purpura (TTP), antiphospholipid syndrome (APS), and complement-mediated TMA.
• The use of caplacizumab in patients with TTP is supported by the guideline, which suggests that it may be used in combination with plasma exchange and immunosuppressive therapy to manage the condition.
• However, the guideline does not provide a specific recommendation for when to discontinue caplacizumab based on ADAMTS13 activity levels, but it does emphasize the importance of monitoring ADAMTS13 activity and adjusting treatment accordingly.
• In clinical practice, the decision to discontinue caplacizumab should be based on the individual patient's response to treatment and the normalization of ADAMTS13 activity, as well as the presence of other risk factors for TTP recurrence.
• The most recent and highest quality study on this topic is the KDIGO 2024 guideline, which provides a comprehensive approach to the management of lupus nephritis and TMA 1.

From the Research

Stopping Caplacizumab Based on ADAMTS13

• The decision to stop caplacizumab based on ADAMTS13 levels is a complex one, and there is evidence to suggest that it should be individualized 2.
• Studies have shown that caplacizumab is effective in treating thrombotic thrombocytopenic purpura (TTP) by inhibiting the interaction between von Willebrand factor and platelets, and that it can reduce the risk of exacerbations and relapses 3, 4.
• However, caplacizumab has also been associated with an increased risk of bleeding, particularly mucocutaneous bleeding, which can be a significant concern for patients with TTP 4, 5.
• The cost-effectiveness of caplacizumab has also been evaluated, and one study found that it was not cost-effective due to its high cost and failure to improve relapse rates 6.
• In terms of stopping caplacizumab based on ADAMTS13 levels, there is evidence to suggest that it may be possible to discontinue treatment once ADAMTS13 activity has returned to normal, but this decision should be made on a case-by-case basis and in consultation with a healthcare provider 2, 5.
• Key factors to consider when deciding whether to stop caplacizumab based on ADAMTS13 levels include:
  • The patient's individual response to treatment
  • The risk of exacerbations and relapses
  • The risk of bleeding and other adverse events
  • The cost-effectiveness of continued treatment
  • The patient's overall health and well-being 2, 3, 4, 5, 6