Immune Thrombocytopenia (ITP)


Prognosis for patients with immune thrombocytopenia (ITP) varies depending on a number of factors, including disease type, response to treatment, age at disease onset, sex, and extremely low platelet counts resulting in serious bleeding complications that can become life-threatening.1

Overall, the prognosis for both children and adults with ITP is very good, with most patients achieving a complete recovery. The most significant factor influencing prognosis is the risk of serious bleeding that accompanies low platelet counts.2

Effects of ITP Disease Type on Prognosis

Immune thrombocytopenia presents as two distinct types: acute ITP, which lasts less than 6 months, and chronic ITP, which lasts longer than 6 months. Acute ITP occurs predominantly in children, while chronic ITP mostly affects adults.3

Over 80% of untreated children with acute ITP achieve clinical remission spontaneously, making a complete recovery with normal platelet levels within 2 to 8 weeks.1 Acute ITP following precipitating infections in children appears to be benign and self limiting with resolution of symptoms as the body ceases to produce autoantibodies against normal platelets. According to one source, mortality is around 2% in children receiving emergency care for ITP.4

Children with ITP are at higher risk of developing chronic ITP if they1,5

  • are female;
  • are 11 years of age at disease onset or older;
  • test positive for antinuclear antibodies;
  • have an insidious disease onset;
  • have platelet counts of 20,000/µL at initial presentation;
  • have no precipitating infection or vaccination; or
  • were treated with methylprednisolone plus intravenous immunoglobulin (IVIG).

Children demonstrate a lower likelihood of developing chronic ITP if they present with mucosal bleeding at initial diagnosis or receive IVIG treatment alone.1,5

In contrast, only 2% of adults with ITP make a spontaneous recovery, and around 64% eventually recover with treatment. Around 30% of adults with ITP develop chronic ITP, while 5% die from hemorrhage.4

Prognosis is good for those with acute ITP, while those with chronic ITP almost always require treatment and commonly experience relapses.6

Read more about ITP types

ITP Prognosis Associated With Treatment Response

Around 60% to 90% of adults with ITP demonstrate a good response with increased platelet counts following treatment with prednisone or prednisone plus IVIG or intravenous Rho(D) immunoglobulin.1 Approximately 20% of patients with ITP experience a complete response to first-line steroid therapy, while 60% demonstrate a partial response.6 Some patients with ITP experience adverse reactions to corticosteroid treatment.4

Patients with steroid-refractory ITP may require a splenectomy for improved outcomes. Between 50% and 70% of patients demonstrate durable disease remission (either complete or partial responses) after splenectomy.7 The few patients who do not respond to splenectomy may receive vincristine treatment.6 Long-term prognosis, even in refractory cases of ITP, is benign.8

Long-term increased infection risk is one postsplenectomy complication. Pneumococcal infections or severe sepsis following splenectomy may pose a threat to life, especially in elderly patients.4,9 

Read more about ITP treatment

The risk of intracranial hemorrhage is higher in adults with ITP than in children with the disease (1.5% vs ~0.5%), and risk increases with age.10 Advanced age (>60 years) is one of several factors (including severe thrombocytopenia and a previous history of minor bleeding) that predicts more severe bleeding events.10,11

Read more about ITP risk factors

Chronic ITP occurs in women 2 to 3 times more frequently than in men.3 Children have a higher risk of developing chronic ITP if they are female (odds ratio, 1.17).1,5

Read more about ITP epidemiology

Effects of Platelet Counts on ITP Prognosis 

Fatal bleeding occurs during the initial disease presentation in 0.9% of cases.1 Platelet counts lower than 10,000/µL have been associated with an increased risk of serious bleeding, including severe intracranial, gastrointestinal, or vaginal hemorrhage.4,10 The estimated rates of severe bleeding episodes are 3% to 20% among children with ITP and around 10% in adults with ITP.10

The prevention of falls and head injuries to reduce the risk of intracranial hemorrhaging as well as prompt, targeted medical attention in the event of severe bleeding episodes are the most crucial factors affecting ITP prognosis and mortality.2

Read more about ITP complications

References

  1. Kessler CM. Immune thrombocytopenia (ITP): prognosis. Medscape. Updated January 7, 2021. Accessed October 24, 2022.
  2. Idiopathic thrombocytopenic purpura (ITP) causes, symptoms and treatment. Children’s Hospital of Philadelphia. Accessed October 24, 2022.
  3. Immune thrombocytopenia (ITP). National Heart, Lung, and Blood Institute. Updated March 24, 2022. Accessed October 24, 2022.
  4. Silverman MA. Immune thrombocytopenia (ITP) in emergency medicine. Medscape. Updated September 13, 2021. Accessed October 24, 2022.
  5. Heitink-Pollé KMJ, Nijsten J, Boonacker CWB, de Haas M, Bruin MCA. Clinical and laboratory predictors of chronic immune thrombocytopenia in children: a systematic review and meta-analysis. Blood. 2014;124(22):3295-3307. doi:10.1182/blood-2014-04-570127
  6. Justiz Vaillant AA, Gupta N. ITP-immune thrombocytopenic purpura. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated July 8, 2022. Accessed October 24, 2022.
  7. Chaturvedi S, Arnold DM, McCrae KR. Splenectomy for immune thrombocytopenia: down but not out. Blood. 2018;131(11):1172-1182. doi:10.1182/blood-2017-09-742353
  8. Stasi R, Stipa E, Masi M, et al. Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med. 1995;98(5):436-442. doi:10.1016/s0002-9343(99)80342-8
  9. Tahir F, Ahmed J, Malik F. Post-splenectomy sepsis: a review of the literature. Cureus. 2020;12(2):e6898. doi:10.7759/cureus.6898
  10. Arnold DM. Bleeding complications in immune thrombocytopenia. Hematology Am Soc Hematol Educ Program. 2015;2015(1):237-242. doi:10.1182/asheducation-2015.1.237
  11. Cortelazzo S, Finazzi G, Buelli M, Molteni A, Viero P, Barbui T. High risk of severe bleeding in aged patients with chronic idiopathic thrombocytopenic purpura. Blood. 1991;77(1):31-33. doi:10.1182/blood.V77.1.31.31

Reviewed by Kyle Habet, MD, on 10/24/2022.

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