부산대학교 도서관

Objectives To investigate the impact of thrombocytopenia on survival in patients with APS. Methods Thrombocytopenia and other predictors of outcome were retrospectively evaluated in an aPL-positive and APS cohort with 38-year follow-up (1980–2018). Thrombocytopenia was defined as <150 × 109 platelets/l. Hazard ratios (HR) of mortality were calculated using Cox-regression models. Results Among 114 patients, 64% had primary APS, 25% secondary APS and 10% asymptomatic aPL. Mean follow-up was 19 (range 5–38) years. ANA [hazard ratio (HR) 1.8, 95% CI 0.8, 3.6, P  = 0.10], arterial thrombotic events (HR 7.0, 95% CI 1.4, 3.5, P  = 0.016), myocardial infarction (HR 8.3, 95% CI 1.1, 59, P  = 0.03), intracardiac thrombosis (HR 17, 95% CI 1, 279, P  = 0.04) and thrombocytopenia (HR 2.9, 95% CI 1.4, 6.1, P  = 0.004) were risk factors for all-cause mortality, but in multivariate analysis only thrombocytopenia (HR 2.7, 95% CI 1.3, 6.0, P  = 0.01) remained significant. Persistent (HR 4.4, 95% CI 2.1, 9.2, P  = 0.001) and low–moderate thrombocytopenia (HR 2.8, 95% CI 1.2, 6.4, P  = 0.01) were associated with a significant increase in mortality compared with acute (HR 1.6, 95% CI 0.5, 5.3, P  = 0.40) and severe (HR 2.1, 95% CI 0.5, 9.2, P  = 0.30) forms. APS patients with vs without thrombocytopenia were more frequently male (58 vs 24%, P  = 0.001) with arterial thrombosis (55 vs 32%, P  = 0.04), LA positivity (100 vs 87%, P  = 0.04), type I aPL profile (89% vs 71%, P  = 0.05) and anticoagulant treatment (89 vs 63%, P  = 0.01). Thrombosis caused 13% of deaths in thrombocytopenic patients and 1% in those without (P  = 0.01). Conclusion Thrombocytopenia is an aPL-related manifestation that identifies patients with severe disease phenotype and high thrombotic risk. Persistent low–moderate thrombocytopenia is associated with a reduced long-term survival. [ABSTRACT FROM AUTHOR]