Idiopathic Thrombocytopenic Purpura

  • Author: Michael A Silverman, MD, MD; Chief Editor: Gil Z Shlamovitz, MD, FACEP  more...
 
Updated: Jul 22, 2016
 

Background

Idiopathic thrombocytopenic purpura (ITP), also known as primary immune thrombocytopenic purpura and autoimmune thrombocytopenic purpura, is defined as isolated thrombocytopenia with normal bone marrow and in the absence of other causes of thrombocytopenia. ITP has two distinct clinical syndromes, manifesting as an acute condition in children and a chronic condition in adults.

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Pathophysiology

ITP is primarily a disease of increased peripheral platelet destruction, with most patients having antibodies to specific platelet membrane glycoproteins. Relative marrow failure may contribute to this condition, since studies show that most patients have either normal or diminished platelet production.

Acute ITP often follows an acute infection and has a spontaneous resolution within 2 months. Chronic ITP persists longer than 6 months without a specific cause.

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Epidemiology

Frequency

United States

Incidence rates are as follows:

  • The incidence of ITP in adults is approximately 66 cases per 1,000,000 per year
  • An average estimate of the incidence in children is 50 cases per 1,000,000 per year
  • New cases of chronic refractory ITP comprise approximately 10 cases per 1,000,000 per year

International

According to studies in Denmark and England, childhood ITP occurs in approximately 10-40 cases per 1,000,000 per year.[20] A prospective, population-based study in Norway indicated an incidence of 53 per 1,000,000 in children younger than 15 years.[46] A study in Kuwait reported a higher incidence of 125 cases per 1,000,000 per year.

Mortality/Morbidity

Hemorrhage represents the most serious complication; intracranial hemorrhage is the most significant. The mortality rate from hemorrhage is approximately 1% in children and 5% in adults. In patients with severe thrombocytopenia, predicted 5-year mortality rates from bleeding are significantly raised in patients older than 60 years versus patients younger than 40 years, 47.8% versus 2.2%, respectively. Older age and previous history of hemorrhage increase the risk of severe bleeding in adult ITP.

Spontaneous remission occurs in more than 80% of cases in children. However, it is uncommon in adults.

Age

See the list below:

  • Peak prevalence occurs in adults aged 20-50 years.
  • Peak prevalence occurs in children aged 2-4 years.
  • Approximately 40% of all patients are younger than 10 years.
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Sex- and Age-related Demographics

In chronic ITP (adults), the female-to-male ratio is 2.6:1. More than 72% of patients older than 10 years are female. In acute ITP (children), distribution is equal between males (52%) and females (48%).

In adults, the peak prevalence is from 20-50 years of age. In children, peak prevalence is from 2-4 years of age. Approximately 40% of all patients are younger than 10 years.

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Contributor Information and Disclosures
Author

Michael A Silverman, MD, MD Chairman, Department of Emergency Medicine, Virginia Hospital Center; Instructor of Emergency Medicine, Johns Hopkins University School of Medicine

Michael A Silverman, MD, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine, American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Gil Z Shlamovitz, MD, FACEP Associate Professor of Clinical Emergency Medicine, Keck School of Medicine of the University of Southern California; Chief Medical Information Officer, Keck Medicine of USC

Gil Z Shlamovitz, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association

Disclosure: Nothing to disclose.

Additional Contributors

Edward A Michelson, MD Associate Professor, Program Director, Department of Emergency Medicine, University Hospital Health Systems of Cleveland

Edward A Michelson, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

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