eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Idiopathic Thrombocytopenic Purpura

Author: Michael A Silverman, MD, Instructor of Emergency Medicine, The Johns Hopkins University School of Medicine; Chairman, Department of Emergency Medicine, Harbor Hospital
Contributor Information and Disclosures

Updated: Jan 21, 2009

Introduction

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 the absence of other causes of thrombocytopenia. The 2 distinct clinical syndromes manifest as an acute condition in children and a chronic condition in adults.

ITP is a decrease in the number of circulating platelets in the absence of toxic exposure or a disease associated with a low platelet count.

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.

Frequency

United States

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. 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 but is uncommon in adults.

Sex

  • 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%).

Age

  • 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.

Clinical

History

  • Focus on the symptoms of bleeding (eg, type, severity, duration) and on symptoms that may exclude other causes of thrombocytopenia.
  • Elicit risk factors for HIV and systemic symptoms linked to other illnesses or to medications (eg, heparin, alcohol, quinidine/quinine, sulfonamides) that may cause thrombocytopenia.
  • Address risk factors for increased bleeding, such as GI disease, CNS disease, urologic disease, or active lifestyle, as these may determine the aggressiveness of management.
  • Common signs, symptoms, and precipitating factors include the following:
    • Abrupt onset (childhood ITP)
    • Gradual onset (adult ITP)
    • Purpura
    • Menorrhagia
    • Epistaxis
    • Gingival bleeding
    • Recent live virus immunization (childhood ITP)
    • Recent viral illness (childhood ITP)
    • Bruising tendency
  • Limited data are available on the recurrent form of the disease. One study showed a 6% prevalence of recurrent ITP with most patients (69%) having only one recurrence. Though one third of patients had their recurrent episode within 3 months of their initial one, the remainder of patients had at least a 3-month interval between episodes.

Physical

Evaluate the type and the severity of bleeding and try to exclude other causes of bleeding. Seek evidence of liver disease, thrombosis, autoimmune diseases (eg, nephritis, cutaneous vasculitis, arthritis), and infection, particularly HIV.

  • Common physical findings include the following:
    • Nonpalpable petechiae, which mostly occur in dependent regions
    • Hemorrhagic bullae on mucous membranes
    • Purpura
    • Gingival bleeding
    • Signs of GI bleeding
    • Menometrorrhagia, menorrhagia
    • Retinal hemorrhages
    • Evidence of intracranial hemorrhage, with possible neurologic symptoms
    • Nonpalpable spleen: The prevalence of palpable spleen in patients with ITP is approximately the same as that in the non-ITP population (ie, 3% in adults, 12% in children).
    • Spontaneous bleeding when platelet count is less than 20,000/mm3.

Causes

  • Immunoglobulin G (IgG) autoantibodies on the platelet surface

More on Idiopathic Thrombocytopenic Purpura

Overview: Idiopathic Thrombocytopenic Purpura
Differential Diagnoses & Workup: Idiopathic Thrombocytopenic Purpura
Treatment & Medication: Idiopathic Thrombocytopenic Purpura
Follow-up: Idiopathic Thrombocytopenic Purpura
References
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Further Reading

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Keywords

idiopathic thrombocytopenic purpura, ITP, platelets, primary immune thrombocytopenic purpura, autoimmune thrombocytopenic purpura, thrombocytopenia, hemorrhage, acute ITP, childhood ITP, adult ITP, purpura, isolated thrombocytopenia, splenectomy, platelet count, decrease in number of platelets, increased destruction of platelets, chronic refractory ITP, intracranial hemorrhage, bleeding, menorrhagia, epistaxis, gingival bleeding, recent live virus immunization, recent viral illness, bruising tendency, nephritis,cutaneous vasculitis, arthritis, HIV, petechiae, hemorrhagic bullae, menometrorrhagia, retinal hemorrhages, spontaneous bleeding, immunoglobulin G autoantibodies

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Contributor Information and Disclosures

Author

Michael A Silverman, MD, Instructor of Emergency Medicine, The Johns Hopkins University School of Medicine; Chairman, Department of Emergency Medicine, Harbor Hospital
Michael A Silverman, MD is a member of the following medical societies: American College of Emergency Physicians, American College of Physician Executives, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Edward A Michelson, MD, Program Director, Associate Professor, Department of Emergency Medicine, University Hospital Health Systems in Cleveland
Edward A Michelson, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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 and American College of Physicians
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Pamela L Dyne, MD, Professor of Clinical Medicine/Emergency Medicine, David Geffen School of Medicine at UCLA; Attending Physician, Department of Emergency Medicine, Olive View-UCLA Medical Center
Pamela L Dyne, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

 
 
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