Relapse Free Survival Progressive Shortens in a Subset of Black patients with Immune TTP Treated in the Rituximab Era

Immune thrombotic thrombocytopenic purpura (iTTP) is a chronically relapsing disorder caused by autoantibody mediated deficiency of ADAMTS13. Rituximab is frequently administered to prevent relapses, but whether the durability of rituximab is maintained with subsequent treatment courses has not been studied. Using the United States Thrombotic Microangiopathy Consortium (USTMA) retrospective iTTP registry, we evaluated clinical relapse free survival (RFS) with subsequent courses of rituximab treatment in multiply relapsing patients. Separately, we evaluated overall RFS (composite of time to clinical relapse, ADAMTS13 relapse, or preemptive rituximab) in a prospective iTTP cohort from the Johns Hopkins University and the University of Minnesota. In the USTMA registry, median clinical RFS was shorter after the 2nd or subsequent rituximab-treated episode compared to the first (2.1 vs 6.0 years, P = 0.04). White patients' clinical relapse risk after the second and subsequent rituximab courses was not significantly different compared to the first [HR=1.86 (95% CI 0.22-15.80), P=0.57], while for Black patients, clinical relapse risk was significantly higher after the second or subsequent courses [HR=2.82 (95% CI 1.52-5.24), P=0.001]. In the prospective cohort, overall RFS progressively shortened after each episode of rituximab treatment with the first episode having the longest RFS (2.8 years IQR 2.0-6.0) and this loss of response durability was most pronounced in Black patients. The durability of rituximab's effect declines with subsequent treatments, which is more pronounced in Black patients who may benefit from closer monitoring and alternative immunomodulatory approaches such as maintenance rituximab and consideration of other agents.

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UNC Libraries
Published
2024-12-17
DOI
https://doi.org/10.17615/pf72-y335
Primary Topic
Urticaria and Related Conditions
Type
article
Field-Weighted Citation Impact
0.00

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article

Relapse Free Survival Progressive Shortens in a Subset of Black patients with Immune TTP Treated in the Rituximab Era

Michael D. Evans, Ayotola Fatola, J. Evan Sadler, Jay S. Raval et al.
UNC Libraries
Urticaria and Related Conditions
article

Relapse Free Survival Progressive Shortens in a Subset of Black patients with Immune TTP Treated in the Rituximab Era

Michael D. Evans, Ayotola Fatola, J. Evan Sadler, Jay S. Raval, Jenna Brown, Susan Eubanks, Radhika Gangaraju, Ara Metjian, Todd Clover, X. Long Zheng, Yara A. Park, Ronald S. Go, Frank Akwaa, Darla Liles, Keith R. McCrae, Nicole K. Kocher, Ming Y. Lim, Meera Sridharan, Ryan Woods, Shruti Chaturvedi, Gustaaf de Ridder, Andrew M. Farland, Spero R. Cataland, Andrew D. Johnson, Raj S. Kasthuri, Ana G. Antun, Marshall Mazepa, Camila Masias, Lisa Baumann Kreuziger, Harsh Vardhan Upreti, Briana Gibson, Elizabeth E. Davis, USTMA Consortium
article en

Abstract

Immune thrombotic thrombocytopenic purpura (iTTP) is a chronically relapsing disorder caused by autoantibody mediated deficiency of ADAMTS13. Rituximab is frequently administered to prevent relapses, but whether the durability of rituximab is maintained with subsequent treatment courses has not been studied. Using the United States Thrombotic Microangiopathy Consortium (USTMA) retrospective iTTP registry, we evaluated clinical relapse free survival (RFS) with subsequent courses of rituximab treatment in multiply relapsing patients. Separately, we evaluated overall RFS (composite of time to clinical relapse, ADAMTS13 relapse, or preemptive rituximab) in a prospective iTTP cohort from the Johns Hopkins University and the University of Minnesota. In the USTMA registry, median clinical RFS was shorter after the 2nd or subsequent rituximab-treated episode compared to the first (2.1 vs 6.0 years, P = 0.04). White patients' clinical relapse risk after the second and subsequent rituximab courses was not significantly different compared to the first [HR=1.86 (95% CI 0.22-15.80), P=0.57], while for Black patients, clinical relapse risk was significantly higher after the second or subsequent courses [HR=2.82 (95% CI 1.52-5.24), P=0.001]. In the prospective cohort, overall RFS progressively shortened after each episode of rituximab treatment with the first episode having the longest RFS (2.8 years IQR 2.0-6.0) and this loss of response durability was most pronounced in Black patients. The durability of rituximab's effect declines with subsequent treatments, which is more pronounced in Black patients who may benefit from closer monitoring and alternative immunomodulatory approaches such as maintenance rituximab and consideration of other agents.

UNC Libraries
University of North Carolina at Chapel Hill (US)
University of Minnesota, National Institutes of Health, University of North Carolina at Chapel Hill, National Center for Advancing Translational Sciences
Zero hunger
Openalex Percentile: Top 59%
Urticaria and Related Conditions
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