Immune thrombotic thrombocytopenic purpura in older patients: prognosis and long-term survival

• Published in: Blood Vol. 134; no. 24; pp. 2209 - 2217
• Main Authors: Prevel, Renaud, Roubaud-Baudron, Claire, Gourlain, Samuel, Jamme, Matthieu, Peres, Karine, Benhamou, Ygal, Galicier, Lionel, Azoulay, Elie, Poullin, Pascale, Provôt, François, Maury, Eric, Presne, Claire, Hamidou, Mohamed, Saheb, Samir, Wynckel, Alain, Servais, Aude, Girault, Stéphane, Delmas, Yahsou, Chatelet, Valérie, Augusto, Jean-François, Mousson, Christiane, Perez, Pierre, Halimi, Jean-Michel, Kanouni, Tarik, Lautrette, Alexandre, Charvet-Rumpler, Anne, Deligny, Christophe, Chauveau, Dominique, Veyradier, Agnès, Coppo, Paul
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 12.12.2019
• Subjects: Adult; Aged; Aged, 80 and over; Biomarkers; Combined Modality Therapy; Comorbidity; Disease Management; Female; France - epidemiology; Humans; Male; Middle Aged; Outcome Assessment, Health Care; Prognosis; Public Health Surveillance; Purpura, Thrombocytopenic, Idiopathic - diagnosis; Purpura, Thrombocytopenic, Idiopathic - epidemiology; Purpura, Thrombocytopenic, Idiopathic - mortality; Purpura, Thrombocytopenic, Idiopathic - therapy; Registries; Survival Analysis; Symptom Assessment; France
• ISSN: 0006-4971; 1528-0020; 1528-0020
• DOI: 10.1182/blood.2019000748

Older age is associated with increased mortality in immune thrombotic thrombocytopenic purpura (iTTP). Yet, data are scarce regarding iTTP occurring among older patients. To assess clinical features and long-term impact of iTTP on mortality in older patients (>60 years old), characteristics and prognoses of adult iTTP patients enrolled in the French Reference Center for Thrombotic Microangiopathies registry between 2000 and 2016 were described according to age (<60 years old or ≥60 years old). Long-term mortality of iTTP older survivors was compared with that of non-iTTP geriatric subjects. Comparing, respectively, older iTTP patients (N = 71) with younger patients (N = 340), time from hospital admission to diagnosis was longer (P < .0001); at diagnosis, delirium (P = .034), behavior impairment (P = .045), renal involvement (P < .0001), and elevated troponin level (P = .025) were more important whereas cytopenias were less profound (platelet count, 22 × 103/mm3 [9-57] vs 13 × 103/mm3 [9-21], respectively [P = .002]; hemoglobin level, 9 g/dL [8-11] vs 8 g/dL [7-10], respectively [P = .0007]). Short- and mid-term mortalities were higher (P < .0001) and increased for every 10 years of age range. Age ≥60 years, cardiac involvement, increased plasma creatinine level, and total plasma exchange volume were independently associated with 1-month mortality. Compared with a non-iTTP geriatric population, older survivors showed an increased long-term mortality (hazard ratio = 3.44; P < .001). In conclusion, older iTTP patients have atypical neurological presentation delaying the diagnosis. Age negatively impacts short-term but also long-term mortality. •iTTP in older patients has atypical clinical features delaying diagnosis; 1-month and 1-year mortality rates are higher.•A history of iTTP in older patients negatively impacts the life expectancy in survivors. [Display omitted]