eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Immune Thrombocytopenic Purpura

Author: S Gerald Sandler, MD, FACP, FCAP, Professor of Medicine and Pathology; Director, Transfusion Medicine, Department of Laboratory Medicine, Georgetown University Hospital
Coauthor(s): Rumina Bhanji, MD, Staff Physician, Departments of Pathology and Laboratory Medicine, Georgetown University Hospital
Contributor Information and Disclosures

Updated: Oct 4, 2009

Introduction

Background

Immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a decreased number of circulating platelets (thrombocytopenia) manifests as a bleeding tendency, easy bruising (purpura), or extravasation of blood from capillaries into skin and mucous membranes (petechiae).

In persons with immune thrombocytopenic purpura (ITP), platelets are coated with autoantibodies to platelet membrane antigens, resulting in splenic sequestration and phagocytosis by mononuclear macrophages. The resulting shortened life span of platelets in the circulation, together with incomplete compensation by increased platelet production by bone marrow megakaryocytes, results in a decreased platelet count.

To establish a diagnosis of immune thrombocytopenic purpura (ITP), exclude other causes of thrombocytopenia, such as leukemia, myelophthisic marrow infiltration, myelodysplasia, aplastic anemia, or adverse drug reactions. Pseudothrombocytopenia due to platelet clumping is also a diagnostic consideration.

No single laboratory result or clinical finding establishes a diagnosis of immune thrombocytopenic purpura (ITP); it is a diagnosis of exclusion.

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center and Cuts, Scrapes, Bruises, and Blisters Center. Also, see eMedicine's patient education article Bruises.

Pathophysiology

In immune thrombocytopenic purpura (ITP), an abnormal autoantibody, usually immunoglobulin G (IgG) with specificity for 1 or more platelet membrane glycoproteins (GPs), binds to circulating platelet membranes.1,2,3

Autoantibody-coated platelets induce Fc receptor-mediated phagocytosis by mononuclear macrophages, primarily but not exclusively in the spleen.4 The spleen is the key organ in the pathophysiology of immune thrombocytopenic purpura (ITP), not only because platelet autoantibodies are formed in the white pulp, but also because mononuclear macrophages in the red pulp destroy immunoglobulin-coated platelets.5

If bone marrow megakaryocytes cannot increase production and maintain a normal number of circulating platelets, thrombocytopenia and purpura develop. Impaired thrombopoiesis is attributed to failure of a compensatory increase in thrombopoietin and megakaryocyte apoptosis.

Frequency

United States

The annual incidence of immune thrombocytopenic purpura (ITP) is estimated to be 5 cases per 100,000 children and 2 cases per 100,000 adults,6 but these data are not from large population-based studies. Most cases of acute immune thrombocytopenic purpura (ITP), particularly in children, are mild and self-limited and may not receive medical attention. Therefore, estimated incidences of acute immune thrombocytopenic purpura (ITP) are difficult to determine and are likely to understate the full extent of the disease. The age-adjusted prevalence of immune thrombocytopenic purpura (ITP) was reported as 9.5 per 100,000 persons in Maryland by Segal and Powe.7

Mortality/Morbidity

  • Hemorrhage: The primary cause of long-term morbidity and mortality in patients with immune thrombocytopenic purpura (ITP) is hemorrhage.8
  • Intracranial hemorrhage: The most frequent cause of death in association with immune thrombocytopenic purpura (ITP) is spontaneous or accidental trauma-induced intracranial bleeding. Most cases of intracranial hemorrhage occur in patients whose platelet counts are less than 10 X 109/L (<10 X 103/µL).9 This situation occurs in 0.5-1% of children and half are fatal.6 In one study, 17% of children experienced a major hemorrhage.10
  • Treatment-related morbidity: To maintain a platelet count in a safe range in patients with chronic treatment-resistant immune thrombocytopenic purpura (ITP), a long-term course of corticosteroids, other immunosuppressive medications, or splenectomy may be required. In patients with immune thrombocytopenic purpura (ITP), morbidity and mortality can be related to treatment, reflecting the complications of therapy with corticosteroids or splenectomy.

Sex

  • In children, immune thrombocytopenic purpura (ITP) is more common among boys compared with girls.11
  • In middle-aged adults, women are affected more frequently than men.6

Age

  • Children may be affected at any age with immune thrombocytopenic purpura (ITP), but the prevalence peaks in children aged 1-6 years.11
  • Adults may be affected at any age, but most cases are diagnosed in women aged 30-40 years.
  • Onset in a patient older than 60 years is uncommon, and a search for other causes of thrombocytopenia is warranted. The most likely causes in these persons are myelodysplastic syndromes, acute leukemia, and marrow infiltration (myelophthisis).

Clinical

History

The medical history in a patient with a clinical suspicion of immune thrombocytopenic purpura (ITP) should focus on (1) factors that suggest another disease for which thrombocytopenia is a complication12 and (2) signs and symptoms that differentiate mild, moderate, and severe bleeding tendencies.

  • Other systemic illnesses
    • In adults, thrombocytopenic purpura may be a manifestation of systemic lupus erythematosus13 or acute or chronic leukemia.
    • Thrombocytopenic purpura may be a manifestation of a myelodysplastic syndrome, particularly in patients older than 60 years.
    • In young children, immune thrombocytopenic purpura (ITP) may manifest as a primary immune deficiency syndrome.
  • Postviral illness
    • In children, most cases of immune thrombocytopenic purpura (ITP) are acute, and onset seems to occur within a few weeks of recovery from a viral illness. The severity of symptoms of the viral illness is not correlated with the degree of thrombocytopenia.
    • Thrombocytopenia is a recognized complication after infection with Epstein-Barr virus, varicella virus, cytomegalovirus, rubella virus, or hepatitis virus (A, B, or C), although the most typical association is a vaguely defined, viral, upper respiratory infection, or gastroenteritis.
    • Transient thrombocytopenia has been reported to be associated with recent immunization with attenuated live-virus vaccines.14,15
  • Human immunodeficiency virus (HIV) infection
    • Thrombocytopenia may occur during the acute retroviral syndrome coincident with fever, rash, and sore throat.
    • Thrombocytopenia may be a manifestation of acquired immunodeficiency virus syndrome (AIDS), occurring late in the course of HIV infection.
    • Thrombocytopenia not uncommonly marks the onset of symptomatic HIV infection, particularly in people who abuse drugs.
  • Drug-induced thrombocytopenia
    • Regard any medication taken by a person who develops thrombocytopenia as a potential causative agent. A history of all prescription and over-the-counter medications is required to exclude drug-related thrombocytopenia.16
    • Persons who have been sensitized (by previous exposure) to quinidine or quinine may develop immune-mediated drug purpura within hours to days of subsequent exposure. To exclude drug purpura in a person previously treated with quinidine or quinine, the history must include questions about possible exposure to over-the-counter medications, tonic water in cocktails, or bitter lemon beverages.
    • Investigate the records of patients who have been hospitalized and who develop acute thrombocytopenias for all of their medications that are listed and not listed in nursing charts. For example, people who are at risk for heparin-induced thrombocytopenia because of current or recent treatment with heparin may be receiving the heparin with the routine flushing of intravenous (IV) catheters, and this exposure may not be listed on the nursing medication sheet. Many catheters are also heparin impregnated, and unless checked, they can be a hidden cause of heparin-induced thrombocytopenia.
    • Other drugs associated with drug purpura include antibiotics (eg, cephalosporins, rifampicin), gold salts, analgesics, neuroleptics, diuretics, antihypertensives, eptifibatide (Integrilin), and abciximab (ReoPro), which is a Fab fragment of the chimeric human-murine monoclonal antibody 7E3 directed against the platelet GPIIb/IIIa receptor.
    • Acute and chronic alcohol consumption may also be associated with thrombocytopenia. In persons with chronic liver disease, hypersplenism with secondary thrombocytopenia is not uncommon.
  • Bleeding tendency
    • Determine the extent and duration of the bleeding tendency to estimate the severity of the illness and the potential risk for a serious hemorrhage. Previous surgical history can often provide a useful clue regarding the acuteness of thrombocytopenia.
    • Query patients to elicit signs or symptoms of intracranial bleeding, such as headache, blurred vision, somnolence, or loss of consciousness.
    • Patients should report any recent accidental head trauma.
    • Record any bleeding, including petechiae, ecchymoses, epistaxis, menorrhagia, melena, or hematuria. Determine if bruising or bleeding is a recurrent problem.

Physical

Similar to the medical history, focus the physical examination on (1) findings that suggest another disease for which thrombocytopenia is a complication and (2) physical signs that suggest serious internal bleeding.

  • General health
    • Immune thrombocytopenic purpura (ITP) is a primary illness occurring in an otherwise healthy person.
    • Signs of chronic disease, infection, wasting, or poor nutrition indicate that the patient has another illness.
  • Vital signs: Hypertension and bradycardia may be signs of increased intracranial pressure and evidence of an undiagnosed intracranial hemorrhage.
  • Skin and mucous membranes
    • An initial impression of the severity of immune thrombocytopenic purpura (ITP) is formed by examining the skin and mucous membranes.
    • Widespread petechiae and ecchymoses, oozing from a venipuncture site, gingival bleeding, and hemorrhagic bullae indicate that the patient is at risk for a serious bleeding complication. If the patient's blood pressure was taken recently, petechiae may be observed under and distal to the area where the cuff was placed and inflated. Suction-type electrocardiograph (ECG) leads may similarly induce petechiae.
    • Mild thrombocytopenia and a relatively low risk for a serious bleeding complication may manifest as petechiae over the ankles in patients who are ambulatory or on the back in patients who are bedridden.
  • Cardiovascular system: Distant low-amplitude heart sounds accompanied by jugular venous distention may be evidence of hemopericardium.
  • Abdomen
    • In children with acute immune thrombocytopenic purpura (ITP), the presence of a readily palpable spleen is not typical.
    • In an adult, hepatosplenomegaly is also atypical for immune thrombocytopenic purpura (ITP) and may indicate chronic liver and other diseases. In fact, splenomegaly excludes the diagnosis of immune thrombocytopenic purpura (ITP).
  • Nervous system
    • Any asymmetrical finding of recent onset can indicate an intracranial hemorrhage.
    • Pupils should be equal in size and have intact extraocular muscles and symmetrical eye movements.
    • Balance and gait should be intact.
    • Funduscopic examination reveals whether the margins of the optic disc are blurred. Examine the patient for the presence of retinal hemorrhages and other evidence of increased intracranial pressure.

Causes

In children, most cases of immune thrombocytopenic purpura (ITP) are acute, manifesting a few weeks after a viral illness. In adults, most cases of immune thrombocytopenic purpura (ITP) are chronic, manifesting with an insidious onset, typically in middle-aged women. These clinical presentations suggest that the triggering events may be different. However, in both children and adults, the cause of thrombocytopenia (destruction of [antibody-coated] immunoglobulin-coated platelets by mononuclear macrophages) appears to be similar.

  • Autoantibody stimulation
    • In persons with chronic immune thrombocytopenic purpura (ITP), membrane GPs on the surface of platelets become immunogenic, stimulating the production of platelet autoantibodies.
    • In persons with acute immune thrombocytopenic purpura (ITP), the stimulus for autoantibody production is also unknown. Platelet membrane cryptantigens may become exposed by the stress of infection, or pseudoantigens may be formed by the passive adsorption of pathogens on platelet surfaces.
  • Autoantibody specificity
    • In persons with chronic immune thrombocytopenic purpura (ITP), approximately 75% of autoantibodies are directed against platelet GPIIb/IIIa or GPIb/IX GP complexes.
    • Presumably, the remaining 25% are directed against other membrane epitopes, including GPV, GPIa/IIa, or GPIV.
  • Role of the spleen5
    • The spleen is the site of autoantibody production (white pulp).
    • It is also the site of phagocytosis of autoantibody-coated platelets (red pulp).
    • The slow passage of platelets through splenic sinusoids with a high local concentration of antibodies and Fc-gamma receptors on splenic macrophages lend to the uniqueness of the spleen as a site of platelet destruction.
    • Low-affinity macrophage receptors, Fc gamma RIIA, and Fc gamma RIIIA bind immune-complexed IgG and are the key mediators of platelet clearance.
  • Platelet destruction
    • The mononuclear macrophage system of the spleen is responsible for removing platelets in immune thrombocytopenic purpura (ITP), because splenectomy results in prompt restoration of normal platelet counts in most patients with immune thrombocytopenic purpura (ITP).
    • Platelets are sequestered and destroyed by mononuclear macrophages, which are neither reticular nor endothelial in origin. Therefore, the former designation of reticuloendothelial system is considered imprecise.
    • Immune destruction of immunoglobulin-coated platelets is mediated by macrophage IgG Fc (Fc gamma RI, Fc gamma RII, and Fc gamma RIII) and complement receptors (CR1, CR3).

More on Immune Thrombocytopenic Purpura

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

Related eMedicine Topics

Clinical Trial

Clinical Guideline

  • Thrombocytopenia. Finnish Medical Society Duodecim - Professional Association. 2001 Apr 30 (revised 2007 Apr 27). Various pagings. NGC:005827

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Keywords

immune thrombocytopenic purpura, idiopathic thrombocytopenic purpura, ITP, autoimmune thrombocytopenic purpura, primary thrombocytopenic purpura, thrombocytopenia, purpura, petechiae, thrombocytopenic purpura, bleeding disorder, platelet disorder, splenectomy, intravenous immune globulin therapy, IVIG therapy, IV RhIG therapy, intravenous RhIG therapy, intravenous Rho immune globulin therapy

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Contributor Information and Disclosures

Author

S Gerald Sandler, MD, FACP, FCAP, Professor of Medicine and Pathology; Director, Transfusion Medicine, Department of Laboratory Medicine, Georgetown University Hospital
S Gerald Sandler, MD, FACP, FCAP is a member of the following medical societies: American Association of Blood Banks, College of American Pathologists, International Society of Blood Transfusions, and Medical Society of the District of Columbia
Disclosure: Nothing to disclose.

Coauthor(s)

Rumina Bhanji, MD, Staff Physician, Departments of Pathology and Laboratory Medicine, Georgetown University Hospital
Disclosure: Nothing to disclose.

Medical Editor

Michael Paul Kosty, MD, Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic
Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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