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Persistent ADAMTS13 inhibitor delays recovery of ADAMTS13 activity in caplacizumab-treated Japanese patients with iTTP.
- Source :
-
Blood advances [Blood Adv] 2024 May 14; Vol. 8 (9), pp. 2151-2159. - Publication Year :
- 2024
-
Abstract
- Abstract: For patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP), caplacizumab, a nanobody against von Willebrand factor A1 domain, has become crucial. Delayed normalization of ADAMTS13 activity during caplacizumab therapy has been identified. In a retrospective analysis, we compared platelet count, ADAMTS13 activity, its inhibitor, and anti-ADAMTS13 immunoglobulin G (IgG) levels in acute iTTP cases treated with caplacizumab (n = 14) or without it (n = 16). The median time from initial therapeutic plasma exchange (TPE) to the first rituximab administration was 12 days in the caplacizumab group (n = 11) and 10 days in the group without caplacizumab (n = 13). We evaluated ADAMTS13-related parameters at onset and once a week until day 28 after the first TPE. The number of days until the platelet counts reached ≥150 × 109/L was significantly shorter in the caplacizumab group than in the non-caplacizumab group. The median ADAMTS13 activity levels on days 14, 21, and 28 were significantly lower in the caplacizumab group. The median titers of the ADAMTS13 inhibitor and anti-ADAMTS13 IgG on the same days were significantly higher in the caplacizumab group. Furthermore, the median number of days from the first TPE until finally achieving an ADAMTS13 activity of ≥10% was significantly longer in the caplacizumab group than in the non-caplacizumab group (42 vs 23 days, P = .014). We observed delayed ADAMTS13 activity recovery and continued inhibitor and anti-ADAMTS13 IgG detection in patients with acute iTTP on caplacizumab, possibly because of the decreased number of TPEs and delayed frontline rituximab.<br /> (© 2024 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)
- Subjects :
- Humans
Male
Female
Middle Aged
Retrospective Studies
Adult
Aged
Immunoglobulin G blood
Platelet Count
Japan
Rituximab therapeutic use
Rituximab pharmacology
Treatment Outcome
Plasma Exchange
East Asian People
ADAMTS13 Protein metabolism
Single-Domain Antibodies therapeutic use
Single-Domain Antibodies pharmacology
Purpura, Thrombotic Thrombocytopenic drug therapy
Purpura, Thrombotic Thrombocytopenic therapy
Subjects
Details
- Language :
- English
- ISSN :
- 2473-9537
- Volume :
- 8
- Issue :
- 9
- Database :
- MEDLINE
- Journal :
- Blood advances
- Publication Type :
- Academic Journal
- Accession number :
- 38386976
- Full Text :
- https://doi.org/10.1182/bloodadvances.2023012451