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Multicenter Study
. 2022 Feb 22;6(4):1264-1270.
doi: 10.1182/bloodadvances.2020004169.

Cardiovascular disease is a leading cause of mortality among TTP survivors in clinical remission

Senthil Sukumar  1 , Max Brodsky  2 , Sarah Hussain  3 , Lisa Yanek  4 , Alison Moliterno  2   3 , Robert Brodsky  2   3 , Spero R Cataland  1 , Shruti Chaturvedi  2   3
Affiliations
Multicenter Study

Cardiovascular disease is a leading cause of mortality among TTP survivors in clinical remission

Senthil Sukumar et al. Blood Adv. .

Abstract

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) survivors experience high rates of adverse health sequelae and increased mortality over long-term follow-up. We conducted this multicenter cohort study to evaluate long-term mortality and causes of death in iTTP survivors. Between 2003 and 2020, 222 patients were enrolled in the Ohio State University and Johns Hopkins TTP registries and followed for a median of 4.5 (interquartile range [IQR], 75 0.4-11.5) years. Nine patients died during their first iTTP episode, and 29 patients died during follow-up. Mortality rate was 1.8 times higher than expected from an age-, sex-, and race-adjusted reference population. Cardiovascular disease was a leading primary cause of death (27.6%) tied with relapsed iTTP (27.6%), followed by malignancy (20.7%), infection (13.8%), and other causes (10.3%). Male sex (hazard ratio [HR], 3.74; 95% confidence interval [CI], 1.65-8.48), increasing age (HR, 1.04; 95% CI, 1.01-1.07), and number of iTTP episodes (HR, 1.10; 95% CI, 1.01-1.20) were associated with mortality in a model adjusted for African American race (HR, 0.70; 95% CI, 0.30-1.65), hypertension (HR, 0.47; 95% CI, 0.20-1.08), chronic kidney disease (HR 1.46; 95% CI, 0.65-3.30), and site (HR, 1.46; 95% CI, 0.64-3.30). There was a trend toward shorter survival in patients with lower ADAMTS13 activity during remission (P = .078). Our study highlights the need for survivorship care and investigation focused on cardiovascular disease and early mortality in TTP survivors.

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Figures

None
Graphical abstract
Figure 1.
Figure 1.
Flowchart of individuals included in the analysis. A total of 222 individual patients were treated for acute iTTP at the Johns Hopkins Hospital or OSU, of whom 9 died during the index iTTP episode. The remaining 213 patients were including in the analysis of mortality after an episode of acute iTTP. A subset of 98 patients had available measurements of ADAMTS13 activity in remission and were included the analysis evaluating the association of reduced ADAMTS13 activity in remission with mortality.
Figure 2.
Figure 2.
Mortality rate in patients surviving their first episode of iTTP was higher than the expected mortality rate from an age-, sex-, and race-standardized reference US population. Mortality rate is expressed as deaths per 100 000 person years. AA, African American.
Figure 3.
Figure 3.
Overall survival in patients with most recent remission ADAMTS13 >70% vs ≤70%.
Figure 4.
Figure 4.
Causes of death in patients who survived their first iTTP episodes. Data from our combined cohort (29 deaths) are compared with previously published data from the Oklahoma TTP registry (16 deaths). Cardiovascular disease was a leading cause of death in both cohorts.
See this image and copyright information in PMC

References

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