Background: Population-based cohorts of immune thrombocytopenia (ITP) are useful for understanding occurrence, clinical characteristics and long-term clinical course. This paper describes the content of the Nordic Country Patient Registry for Romiplostim (NCPRR) and provides prevalence and incidence estimates of chronic ITP (cITP).
Methods: The NCPRR, a cohort study established in 2009, includes all adult (≥ 18 years) patients in Denmark, Sweden and Norway with cITP (defined as ITP lasting > 12 months and platelet count < 100 × 109/L), combining data from national health registries and medical records. The NCPRR currently includes prevalent cITP patients diagnosed before 2009 and incident cITP patients diagnosed during 2009-2016. The registry obtains clinical information for cITP patients, including comorbidities, treatments, laboratory values, and complete follow-up for various outcomes.
Findings: The NCPRR currently includes 3831 patients with cITP (1258 prevalent; 2573 incident). In 2009, the prevalence of registered cITP was 10 · 0/100,000 (95%CI:9 · 1-11 · 0) adult persons in Denmark and 10 · 7/100,000 (95% CI: 9 · 9-11 · 4) adults in Sweden. During 2009-2016, the incidence rates of cITP per 100,000 person-years were 2 · 8 (95%CI: 2 · 6-3 · 0), 1 · 8 (95%CI: 1 · 7-1 · 9) and 2 · 1 (95%CI: 1 · 9-2 · 2) in Denmark, Sweden and Norway, respectively. Fifty-eight percent of cITP patients were women. At NCPRR inclusion, 30.2% were aged ≥ 70 years, 23% had a platelet count < 50 × 109/L, 17.4% were splenectomized, 41% had prior ITP therapy, and 8.6% had severe comorbidity.
Interpretation: The NCPRR provides population-based data on the epidemiology and characteristics of almost 4000 cITP patients and is a valuable resource for research.
Funding: This study was partly funded by a research grant from Amgen to Aarhus University.
Keywords: Bone marrow fibrosis; Epidemiology; Immune thrombocytopenia; Incidence; Population-based; Prevalence; Registry.
© 2019 Published by Elsevier Ltd.