Older patients with immune thrombotic thrombocytopenic purpura (iTTP) more often have an atypical neurological presentation, which could result in a delayed diagnosis, according to authors of a recent retrospective analysis.
“Practitioners should be aware of this in order to shorten the time to treatment, which could improve the prognosis in older iTTP patients,” Paul Coppo, MD, PhD, of Hôpital Saint-Antoine, Paris, and coauthors wrote in Blood.
The older patients also had increased 1-month and 1-year mortality compared with younger patients, and had more than a threefold risk of long-term mortality compared with elderly patients without iTTP, according to the study report.
The analysis included 411 patients with iTTP entered into a national registry in France between 2000 and 2016. Seventy-one patients were 60 years of age or older.
Time from hospital admission to diagnosis was 3 days for those older patients, versus just 1 day for patients under 60 years of age (P = .0001), Dr. Coppo and colleagues reported.
Clinical records were available for 67 of the older iTTP patients, of whom 17 had no evidence of delayed diagnosis. The remainder had a “possible diagnostic delay,” according to the report; among those, the iTTP diagnosis was preceded by neurological manifestations in 26 cases, and transient ischemic stroke that usually led to focal deficiency or aphasia in 14 cases. Other features preceding the diagnosis included malaise, behavioral abnormalities, seizure, and dizziness.
Many of these findings are “not specific to a disease, and they are less alarming than in young patients,” the researchers wrote. “In this context, the presence of a thrombocytopenia with anemia should alert physicians to this possible rare diagnosis.”
Older patients also presented with less pronounced cytopenias compared with younger patients, which could have contributed to a late diagnosis, they added.
Older age is a known risk factor for mortality related to iTTP. In the present study, rates of 1-month mortality were 37% for patients aged 60 years and older, and 9% for those younger than age 60 (P less than .0001). The 1-year mortality rates were 49% and 11% for older and younger patients, respectively (P less than .0001).
Compared with older individuals without iTTP from a different study, older iTTP patients had a lower long-term survival rate. iTTP remained an independent risk factor for death even after adjustment for age, sex, and some comorbidities (hazard ratio, 3.44; 95% confidence interval, 2.02-5.87).
The study was partly funded by a grant from the French Ministry of Health. Dr. Coppo reported that he is a clinical advisory board member for Alexion, Ablynx (now part of Sanofi), Shire, and Octapharma. Two other co-authors reported participating in advisory boards for Ablynx.
SOURCE: Prevel R et al. Blood. 2019 Sep 17. doi: 10.1182/blood.2019000748.