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508 Fatigue and Cognition During Remission in Adults with Immune Thrombotic Thrombocytopenic Purpura

Program: Oral and Poster Abstracts
Type: Oral
Session: 904. Outcomes Research—Non-Malignant Conditions: Classical Hematology: From Horses to Zebras
Hematology Disease Topics & Pathways:
Research, epidemiology, Clinical Research, health outcomes research, patient-reported outcomes, survivorship
Sunday, December 11, 2022: 10:15 AM

Shannon M Reeves, BS, MS1*, Amanda J Llaneza, MPH2*, Sara K. Vesely, PhD, MPH3, Janna Journeycake, MD, MSCS4, Mohamad O. Khawandanah, MD5, Marshall Mazepa, MD6, Frank Akwaa, MD7*, Adam Cuker, MD, MS8, Shruti Chaturvedi, MBBS9, Neil Zakai10, Ming Yeong Lim, MBBChir11, Radhika Gangaraju, MD12, San Keller, PhD, MS13*, Spero Cataland, MD14 and Deirdra R Terrell, PhD15

1Univ of Oklahoma Health Sciences Ctr, Oklahoma City, OK
2University of Oklahoma Health Sciences Center, Oklahoma City, OK
3Biostatistics and Epidemiology, Hudson College of Public Health, The Univ. of OK Health Sciences Ctr., Oklahoma City, OK
4Jimmy Everest Center for Cancer and Blood Disorders, University of Oklahoma Health Sciences Center, Oklahoma City, OK
5Stephenson Cancer Center, University of Oklahoma Health Sciences Center, Oklahoma City, OK
6Division of Hematology, Oncology, and Transplantation, University of Minnesota, Minneapolis, MN
7Wilmot Cancer Institute, Division of Hematology and Oncology, University of Rochester Medical Center, Rochester, NY
8Department of Medicine and Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA
9Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD
10Division of Hematology and Oncology, Larner College of Medicine at the University of Vermont, Burlington, VT
11Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, University of Utah, Salt Lake City, UT
12Institute for Cancer Outcomes and Survivorship, University Of Alabama, Birmingham, AL
13American Institutes for Research, Chapel Hill, NC
14Division of Hematology, Department of Internal Medicine, Ohio State University Medical Center, Columbus, OH
15University of Oklahoma Health Sciences Ctr, Oklahoma City, OK

Introduction: Although impaired cognitive function following recovery from immune-mediated thrombotic thrombocytopenic purpura (iTTP) has been well-described, the issue of fatigue is rarely addressed. In our qualitative studies, people with iTTP described fatigue significantly impacted their daily lives. It is unknown if the prevalence of fatigue in iTTP patients differs from the general population. The first aim of this study was to compare fatigue and cognitive scores of people with iTTP in clinical remission to both the United States (US) population and to people with other disorders. Our second aim was to describe clinical severity of fatigue and cognitive impairment among people with iTTP in remission.

Methods: Patients were recruited from 9 US centers (8/2019 - 11/2021). Eligibility included: 1) age >18 years, 2) ADAMTS13 deficiency (<10% activity) at diagnosis or during a relapse and 3) being >1-year from the last episode. Fatigue and cognitive function were assessed with the validated NIH Patient-Reported Outcomes Measurement Information System (PROMIS) measures. PROMIS measures are disease cross-cutting instruments which allow for comparisons to not only the US population but also other disorders. Demographic information was also obtained.

A one sample t-test was used to compare mean fatigue score of iTTP patients to the US population. iTTP patients were compared to other disorders using PROMIS mean scores and 95% confidence intervals (CI). To aid in clinical interpretation of PROMIS scores, severity thresholds (‘No/Mild’, ‘Moderate’, ‘Severe’) were created utilizing T-score ranges as described previously (Terrell DR, et al Blood 2021; 834). Patients were compared by severity categories and clinical characteristics.

Results: 101 patients completed the study (83% female; 52% White; 36% Black; median age 49.5 years [range 26-85 years]). The mean fatigue score in the iTTP patients was 58 (95% CI 56.4, 60.3), significantly worse than the US population (mean 50), (p<0.0001) with higher scores indicating worse function. Comparisons to other disorders indicated that fatigue in iTTP patients was worse than breast cancer survivors and persons who had recovered from severe COVID-19, but similar to patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (Figure 1). The mean cognitive function score in iTTP patients was 45 (95% CI 43.2, 46.8), significantly worse than the US population (p<0.0001) with lower scores indicating worse function. iTTP cognitive function was worse than breast cancer patients and persons who have recovered from severe COVID-19, but similar to patients with SLE (Figure 1).

Table 1 shows the overlap of clinical severity for cognitive and fatigue symptoms. 13% (13/101) of patients in remission had scores indicating ‘Severe’ impairment on both domains (cognitive and fatigue). Notably, the majority (62%) of patients with severe impairment on both measures were > 5 years since their last episode while only 38% had experienced a relapse. In contrast, 36% of patients with ‘No/Mild’ impairment on both measures were > 5 years from their last episode and 50% of them had relapsed. Yet, patients with moderate severity on both measures were the most likely to report surviving a relapse (68%) and 50% of these patients were > 5 years from their most recent episode.

Conclusion: Fatigue and cognitive function in iTTP patients is worse than the US population but similar to chronic autoimmune disorders such as SLE. Further, 62% of people with severe impairment on both fatigue and cognitive measures were more than 5-years from their most recent episode, and a history of disease relapse was not associated with more severe symptoms. These data suggest that even in long-term ‘remission’, people with iTTP continue to struggle with severe fatigue and cognition, and whether this relates to the initial disease occurrence or continued sub-clinical disease activity is unknown. Strengths of this study included the large number of patients that were recruited from 9 US academic centers. Future studies should evaluate the impact of ADAMTS13 activity and subclinical microangiopathy on symptom clinical severity.

Disclosures: Akwaa: Sobi: Research Funding. Cuker: Synergy: Consultancy; New York Blood Center: Consultancy. Chaturvedi: UCB: Honoraria; Argenx: Honoraria; Takeda: Honoraria; Sanofi Genzyme: Honoraria. Lim: Dova: Consultancy; Takeda: Consultancy; Hema Biologics: Consultancy; Forma Therapeutics: Consultancy. Gangaraju: Sanofi Genzyme: Consultancy; Alexion: Consultancy; NHLBI: Research Funding; ASH Scholar Award: Research Funding; Centers for Disease Control and Prevention: Research Funding. Cataland: Takeda: Consultancy, Research Funding; Alexion: Consultancy, Research Funding; Sanofi: Consultancy, Research Funding. Terrell: Takeda: Other: Patient Advisory Board; Sanofi: Consultancy.

*signifies non-member of ASH