Elderly patients with immune thrombotic thrombocytopenic purpura (iTTP) are at an increased risk for mortality in both the short and long term, according to research published in Blood. Furthermore, the atypical clinical presentation associated with iTTP in the elderly population may delay diagnosis.

There is evidence that iTTP is linked with increased early mortality among older patients, and 17% of patients with iTTP are older than 60 years.

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For this study, researchers evaluated data from 411 patients to determine disease characteristics particular to older patients. Of the included patients, 340 were younger than 60 years and 71 were 60 years or older. Patients aged at least 60 years were more likely to have cancer (11% vs 0.3%; P <.0001), diabetes mellitus (21% vs 4%; P <.0001), dyslipidemia (44% vs 9%; P <.0001), and hypertension (65% vs 12%; P <.0001); younger patients, however, were more likely to have autoimmune disease (30% vs 17%) and to use tobacco (40% vs 22%).

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Older patients were more likely to present with delirium and behavioral disturbance compared with younger patients, and diagnosis was generally delayed in this group (3 days vs 1 day; P <.0001). Younger patients were more likely to present with thrombocytopenia and anemia.

Mortality in the short- and long-term was higher among older patients, with 26 of the 71 elderly patients dying within 1 month compared with 32 of the 340 younger patients. Compared with the general elderly population, older patients with iTTP who survived for at least 1 month after diagnosis had a hazard ratio for long-term mortality of 3.44 (P <.001).

History of tobacco use was carried a hazard ratio for long-term mortality of 1.49 (P =.01).

“Older patients with iTTP have a frequent atypical neurological presentation, which may delay the diagnosis,” the researchers wrote. “Practitioners should be aware of this in order to shorten the time to treatment, which could improve the prognosis in older iTTP patients.”


1.     Prevel R, Roubaud-Baudron C, Gourlain S, et al. Immune thrombotic thrombocytopenic purpura in older patients: prognosis and long-term survival [published online December 12, 2019]. Blood. doi:10.1182/blood.2019000748