Abstract

AbstractFew data exist on the long-term prognosis of patients with chronic primary chronic immune thrombocytopenia (ITP). We examined the risk of infections, hemorrhage resulting in hospitalization, hematologic malignancies, and total and cause-specific mortality among patients with ITP compared with the general population. We used population-based medical databases to identify 407 patients with primary chronic ITP diagnosed during 1996 to 2007 and 4069 general population members individually matched on age, sex, and comorbidity level. We used Cox regression analysis to estimate rate ratios (RRs) adjusted for age (≤ 60 or > 60 years), sex, calendar year, and level of comorbidity. The adjusted 1-year RR of infection was 4.5 (95% confidence interval [CI], 3.3-6.1) for patients with chronic ITP compared with the general population cohort. The adjusted RR decreased to 1.8 (95% CI, 1.3-2.5) for the second to fifth year of follow-up. The adjusted 5-year RR was 3.2 (95% CI, 1.2-9.0) for hospitalized intracranial hemorrhage, 4.4 (95% CI, 2.3-8.3) for other hospitalized hemorrhages, and 4.7 (95% CI, 1.7-12.7) for hematologic malignancy. The 5-year all-cause mortality RR was 2.3 (95% CI, 1.8-3.0). In summary, primary chronic ITP was associated with substantially increased long-term risk of infections, hemorrhagic episodes requiring hospitalization, hematologic malignancies, and mortality.

Highlights

  • Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by immune-mediated platelet destruction and suppressed platelet production.[1]

  • To explore long-term clinical outcomes among patients with chronic ITP, we examined rates of infections, bleeding episodes resulting in hospitalization, incident hematologic malignancies, and total and cause-specific mortality among Danish patients with primary chronic ITP diagnosed from January 1, 1996, through July 31, 2007, and compared these rates with those of a matched comparison cohort drawn from the Danish general population

  • 5.1% among members of the comparison cohort, yielding an adjusted mortality rate ratios (MRRs) of 1.3. In this nationwide population-based study we found a substantially increased risk of infections and hemorrhagic episodes in patients with chronic ITP than in the general population

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Summary

Introduction

Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by immune-mediated platelet destruction and suppressed platelet production.[1] In adults, ITP is most often a chronic disease with insidious onset.[2] Until recently, a diagnosis of chronic ITP required persistence of thrombocytopenia for Ն 6 months after diagnosis. In 2000, Cohen et al conducted a pooled analyses that was based on 17 case series encompassing a total of 1817 patients with ITP5; the risk of fatal hemorrhage was estimated to range from 0.4% per year for patients Ͻ 40 years to 13% per year for patients Ն 60 years

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