Race, rituximab, and relapse in TTP

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by recurring episodes of thrombotic microangiopathy, causing ischemic organ impairment. Black patients are overrepresented in iTTP cohorts in the United States, but racial disparities in iTTP outcome and response to therapy have not been studied. Using the United States Thrombotic Microangiopathies Consortium iTTP Registry, we evaluated the impact of race on mortality and relapse-free survival (RFS) in confirmed iTTP in the United States from 1995 to 2020. We separately examined the impact of rituximab therapy and presentation with newly diagnosed (de novo) or relapsed iTTP on RFS by race. A total of 645 participants with 1308 iTTP episodes were available for analysis. Acute iTTP mortality did not differ by race. When all episodes of iTTP were included, Black race was associated with shorter RFS (hazard ratio [HR], 1.60; 95% CI, 1.16-2.21); the addition of rituximab to corticosteroids improved RFS in White (HR, 0.37; 95% CI, 0.18-0.73) but not Black patients (HR, 0.96; 95% CI, 0.71-1.31). In de novo iTTP, rituximab delayed relapse, but Black patients had shorter RFS than White patients, regardless of treatment. In relapsed iTTP, rituximab significantly improved RFS in White but not Black patients. Race affects overall relapse risk and response to rituximab in iTTP. Black patients may require closer monitoring, earlier retreatment, and alternative immunosuppression after rituximab treatment. How race, racism, and social determinants of health contribute to the disparity in relapse risk in iTTP deserves further study.

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Publication Details

Journal
UNC Libraries
Published
2025-04-22
DOI
https://doi.org/10.17615/zxsw-tk37
Primary Topic
Genomics and Rare Diseases
Type
article
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article

Race, rituximab, and relapse in TTP

Michael D. Evans, J. Evan Sadler, Jay S. Raval, Radhika Gangaraju et al.
UNC Libraries
Genomics and Rare Diseases
article

Race, rituximab, and relapse in TTP

Michael D. Evans, J. Evan Sadler, Jay S. Raval, Radhika Gangaraju, Ara Metjian, Todd Clover, X. Long Zheng, Yara A. Park, Ronald S. Go, Frank Akwaa, Darla Liles, Keith R. McCrae, 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, Angela Liu, Briana Gibson, Elizabeth E. Davis, United States Thrombotic Microangiopathies Consortium
article en

Abstract

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by recurring episodes of thrombotic microangiopathy, causing ischemic organ impairment. Black patients are overrepresented in iTTP cohorts in the United States, but racial disparities in iTTP outcome and response to therapy have not been studied. Using the United States Thrombotic Microangiopathies Consortium iTTP Registry, we evaluated the impact of race on mortality and relapse-free survival (RFS) in confirmed iTTP in the United States from 1995 to 2020. We separately examined the impact of rituximab therapy and presentation with newly diagnosed (de novo) or relapsed iTTP on RFS by race. A total of 645 participants with 1308 iTTP episodes were available for analysis. Acute iTTP mortality did not differ by race. When all episodes of iTTP were included, Black race was associated with shorter RFS (hazard ratio [HR], 1.60; 95% CI, 1.16-2.21); the addition of rituximab to corticosteroids improved RFS in White (HR, 0.37; 95% CI, 0.18-0.73) but not Black patients (HR, 0.96; 95% CI, 0.71-1.31). In de novo iTTP, rituximab delayed relapse, but Black patients had shorter RFS than White patients, regardless of treatment. In relapsed iTTP, rituximab significantly improved RFS in White but not Black patients. Race affects overall relapse risk and response to rituximab in iTTP. Black patients may require closer monitoring, earlier retreatment, and alternative immunosuppression after rituximab treatment. How race, racism, and social determinants of health contribute to the disparity in relapse risk in iTTP deserves further study.

UNC Libraries
University of North Carolina at Chapel Hill (US)
Openalex Percentile: Top 72%
Genomics and Rare Diseases
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