Survival in adult patients with chronic primary and secondary immune thrombocytopenia: A population‐based study

Abstract

Background Few studies have investigated long‐term survival in patients with primary immune thrombocytopenia (pITP). Further, changes in prognosis over the past decades and prognosis of secondary immune thrombocytopenia (sITP) are largely unstudied. Our objectives were to study comorbidity‐adjusted prognostic changes and causes of death in chronic pITP and sITP patients. Study Design/Methods Using nationwide Danish health registries 1980–2016, we identified 1762 patients with chronic pITP (median age 58 (IQR, 37–73) years) and 128 with chronic sITP (median age 59 (IQR, 40–73) years). Patients were age‐sex‐matched to 74,781 general population comparators. Comorbidity was assessed using Charlson Comorbidity Index (CCI). Results Overall median survival was reduced by 5.1 years (95% CI, 0.7–9.4) (p < .001) for pITP and 11.1 years (95% CI, 5.8–16.4) (p < .001) for sITP. 5‐year survival increased from 69% (95% CI, 59–78) in 1980–89 to 80% (95% CI, 75–83) in 2010–16 for pITP, and decreased from 100% (95% CI, 89–98) to 64% (95% CI, 87–91) for sITP. However, numbers were small for sITP. 5‐year survival for pITP with high CCI was 41% (95% CI, 32–49), and 85% (95% CI, 83–87) for low CCI. Bleeding, infection and hematological cancer were relatively frequent causes of death with adjusted subhazard ratios of 3.25 (95% CI, 2.33–4.52), 1.53 (95% CI, 1.08–2.16) and 2.16 (95% CI, 1.12–4.16) in pITP respectively, and 10.52 (95% CI, 1.43–77.36) for hematological cancer in sITP. Conclusions Long‐term survival is reduced in chronic ITP but seems to be improving. Comorbidity and sITP are associated with a poor prognosis.

Expert Report on Immune Thrombocytopenia: Current Diagnostics and Treatment – Recommendations from an Expert Group from Austria, Germany, and Switzerland

“…Despite therapeutic advances, life expectancy of patients with chronic ITP is still significantly reduced [25]. The chief causes are major hemorrhage including CNS bleeding, and infections associated with splenectomy or due to immunosuppressive therapy.…”

Time trends in incidence and prevalence of immune thrombocytopenia: A nationwide cohort analysis

“…This is of interest as Denmark, as most of the western world, has an ageing population, with over 60 years of age being the fastest-growing sector of the population. 15 Prevalence does not plateau as neither incidence, as shown here, nor duration of disease 12 are fixed. Longer life expectancy for ITP patients contributing to increased prevalence may be attributed to improvement in treatment and/or earlier recognition, although further investigation is necessary to determine these relationships.…”

“…In a recent study we showed that survival in cITP is improving, 12 and alongside an ageing population, longer duration of disease and previous trends point towards increasing incidence and prevalence. Hence a contemporary update of frequency measures for adult ITP is necessary.…”

“…Using a retrospective cohort design incidence and prevalence of ITP in the adult population (≥18 years) of Denmark was described from 1 January 1980 to 31 December 2016. All patients with ITP were identified using routinely updated Danish nationwide health registers, as elaborated on by Mannering et al 12 Primary and secondary ITP (pITP/ sITP) diagnoses were defined using validated algorithms based on previously registered diagnoses 13 and patients were deemed chronic if they had two ITP diagnoses with at least 12 months between, as agreed by an international working group. 14 Incidence rates were calculated as 5-year averages, and point prevalence yearly using all living patients as of January 1st, using national census data for the Danish adult population.…”

Expertenreport Immunthrombozytopenie – Aktuelle Diagnostik und Therapie

“…Die Lebenserwartung von Patient*innen mit chronischer ITP ist trotz aller therapeutischer Fortschritte immer noch deutlich reduziert [25]. Schwere Blutungen, inclusive ZNS-Blutungen, und Infektionen durch die immunsuppressive Therapie oder nach Splenektomie sind dafür verantwortlich.…”