Immune Thrombocytopenia (ITP)


Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by an abnormal decrease in platelet count. It is caused by a dysfunctional immune system that leads to a decrease in platelet survival and production.1

Life Expectancy Factors

Immune thrombocytopenia is not life-threatening for most patients, and it has high potential for resolving spontaneously or with treatment. Thus, the life expectancy of most patients with ITP is similar to that of the general population. Acute ITP usually resolves spontaneously in 6 months or less without treatment. Chronic ITP, on the other hand, lasts for many years, but patients can survive for decades with the disease.1

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Life Expectancy According to Patient Age

In most children with ITP, symptoms resolve spontaneously, and those who require intervention or progress to chronic disease usually respond well to treatment. Thus, mortality in pediatric patients with ITP is rare and mainly caused by complications due to catastrophic bleeding, particularly intracranial hemorrhage. Most cases of morbidity and mortality in children with chronic ITP are due to infections or other complications of long-term immunosuppressive treatment.1

Although adults with ITP have lower disease resolution rates than children, most patients can be managed with conventional treatment and reach a stable platelet count with 1 or more therapies. In about 10% of adults, spontaneous remission occurs within the first 6 months.6 Another 33% to 67% of adults reach a stable platelet count with first-line treatments. Some adult patients with refractory ITP require other second-line therapies or need splenectomy. The remaining adult patients have severe refractory disease that is difficult to manage and does not respond to any treatment. This fractional subpopulation has a higher mortality risk than the general population, mainly due to complications from bleeding.1,6,7

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Studies on ITP Life Expectancy

While the life expectancy of most patients with mild and moderate ITP is similar to that of the general population,2 several studies have reported a 1.3- to 2.2-fold increased mortality risk for patients with ITP compared to the general population. This increased mortality risk is predominantly due to the increased infection rate, hemorrhage, cardiovascular disease, and hematological cancer-related mortality.1-4

A study by Portielje et al. showed that patients with refractory ITP who had persistently low platelet counts (below 30,000/µL) had a 4.2-fold increased mortality risk compared to that of the general population. They also found that patients who were reclassified as having secondary ITP within a median follow-up time of 9.4 years had a 6-fold higher mortality than the general population, while patients with primary ITP had a 1.3-fold increased mortality.2

In a long-term follow-up study of patients with chronic ITP, mortality was found to be substantially higher in those who were refractory to splenectomy than in those who were in remission after splenectomy. This high mortality was due to chronic severe thrombocytopenia and continuous bleeding problems that needed emergency treatment and frequent hospitalization.5

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References

  1. Pietras NM, Pearson-Shaver AL. Immune thrombocytopenic purpura. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated May 10, 2022. Accessed October 28, 2022.
  2. Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001;97(9):2549-2554. doi:10.1182/blood.v97.9.2549
  3. Djulbegovic B, Cohen Y. The natural history of refractory idiopathic thrombocytopenic purpura. Blood. 2001;98(7):2282-2283. doi:10.1182/blood.v98.7.2282
  4. Frederiksen H, Maegbaek ML, Nørgaard M. Twenty-year mortality of adult patients with primary immune thrombocytopenia: a Danish population-based cohort study. Br J Haematol. 2014;166(2):260-267. doi:10.1111/bjh.12869
  5. McMillan R, Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood. 2004;104(4):956-960. doi:10.1182/blood-2003-11-3908
  6. Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost. 2015;13(3):457-464. doi:10.1111/jth.12813
  7. Miltiadous O, Hou M, Bussel JB. Identifying and treating refractory ITP: difficulty in diagnosis and role of combination treatment. Blood. 2020;135(7):472-490. doi:10.1182/blood.2019003599

Reviewed by Kyle Habet, MD, on 11/1/2022.

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