II. Epidemiology

  1. Incidence: 10 cases per 100,000
  2. Children comprise 50% of cases
    1. Most common Thrombocytopenia cause in children
    2. Peak onset between ages 2 to 4 years

III. Pathophysiology

  1. IgG Antibody develops against platelet membrane antigen
  2. Acute Idiopathic Thrombocytopenic Purpura
    1. Acute onset follows Viral Exanthem or viral Infection
    2. Occurs in otherwise healthy patients
  3. Chronic Idiopathic Thrombocytopenic Purpura
    1. Insidious onset in patient with immune disorder
    2. More common onset in teenage girls

IV. Causes: Secondary Immune Thrombocytopenic Purpura

  1. Lymphoproliferative Disorders or Myelodysplastic Syndrome
    1. Evaluate in all patients over age 60 years with Immune Thrombocytopenic Purpura
  2. Systemic Lupus Erythematosus
  3. Antiphospholipid Syndrome
  4. Graves Disease
  5. Sarcoidosis
  6. HIV Infection
  7. Epstein-Barr Virus Infection (EBV or mononucleosus)
  8. Cytomegalovirus (CMV)
  9. Varicella-Zoster Virus
  10. Hepatitis C Virus

V. Signs and Symptoms

  1. Purpura
  2. Bleeding complications
    1. Associated with severe Thrombocytopenia (Platelet Count <30,000 per uL)
  3. Mild Splenomegaly in 5 to 10% of cases
  4. Absent signs
    1. No fever, lethargy, pallor or weight loss
    2. No bone or Joint Pain
    3. No Lymphadenopathy
    4. No Hepatomegaly

VI. Labs

  1. Platelet Count drops rapidly

VII. Management: First-Line Management

  1. Corticosteroids
    1. Indicated for severe Thrombocytopenia
      1. Typically indicated with Platelet Count <50,000 per uL (especially <30,000 per uL)
    2. Platelets increase within a week of starting Corticosteroids
    3. Dosing
      1. Methylprednisolone 30 ml/kg/day over 20-30 min up to 1 g/day IV OR
      2. Prednisone 1-1.5 mg/kg orally daily
  2. Intravenous Immune globulin (IV IG)
    1. Dose: 1 g/kg/day for 2-3 days
  3. Rituximab (Rituxan)

VIII. Management: Emergent management

  1. Indications for urgent or emergent management (uncommon)
    1. Serious Hemorrhage
    2. Urgent or emergent surgery required
  2. Treatment
    1. Platelet Transfusion at dosing 2-3 fold greater than usual dose

IX. Management: Refractory cases

  1. Anti-D Immune globulin
    1. May be used in Rh Positive patients
    2. May be considered as alternative to Corticosteroids
  2. Thrombopoietin receptor agonist
  3. Splenectomy
    1. Indicated if corticosterioids and Immunoglobulin Are ineffective at maintaining adequate Platelet Counts
    2. Safe and effective (however subjects patient to lifelong Asplenia risk)
    3. May be preferred in younger patients
    4. Gadenstatter (2002) Am J Surg 184:606-10 [PubMed]

X. Course

  1. Children: Acute Idiopathic Thrombocytopenic Purpura
    1. Less severe in children
    2. Recovery within 6 to 12 months for 80-90% of children
    3. Most cases resolve within weeks
  2. Adults
    1. More chronic, insidious course than for adults

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Ontology: Immune thrombocytopenic purpura (C0398650)

Definition (CHV) bleeding or bruising tendency due to low platelet level
Definition (CHV) bleeding or bruising tendency due to low platelet level
Definition (CHV) bleeding or bruising tendency due to low platelet level
Definition (CHV) bleeding or bruising tendency due to low platelet level
Definition (SNOMEDCT_US) An autoimmune coagulation disorder characterized by isolated thrombocytopenia (a platelet count <100,000/microL), in the absence of any underlying disorder that may be associated with thrombocytopenia.
Definition (SCTSPA) Trastorno autoinmunitario de la coagulación caracterizado por trombocitopenia aislada (recuento de plaquetas <100.000/ul), sin un trastorno subyacente que pueda asociarse con trombocitopenia
Definition (NCI) An autoimmune disorder in which the number of circulating platelets is reduced due to their antibody-mediated destruction. ITP is a diagnosis of exclusion and is heterogeneous in origin.
Definition (NCI_NCI-GLOSS) A condition in which platelets (blood cells that cause blood clots to form) are destroyed by the immune system. The low platelet count causes easy bruising and bleeding, which may be seen as purple areas in the skin, mucous membranes, and outer linings of organs.
Definition (MSH) Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.
Concepts Disease or Syndrome (T047)
MSH D016553
ICD9 287.31
ICD10 D69.3
SnomedCT 191316002, 154825008, 191315003, 267567001, 32273002, 13172003, 234490009
English Autoimmune Thrombocytopenic Purpura, Autoimmune Thrombocytopenic Purpuras, Disease, Werlhof, Idiopathic Thrombocytopenic Purpura, Idiopathic Thrombocytopenic Purpuras, Purpura, Autoimmune Thrombocytopenic, Purpura, Idiopathic Thrombocytopenic, Purpura, Thrombocytopenic, Autoimmune, Purpura, Thrombocytopenic, Idiopathic, Purpuras, Autoimmune Thrombocytopenic, Purpuras, Idiopathic Thrombocytopenic, Thrombocytopenic Purpura, Idiopathic, Thrombocytopenic Purpuras, Idiopathic, Werlhof Disease, Werlhof's Disease, Werlhofs Disease, Disease, Werlhof's, Autoimmune thrombocytopen purp, ITP - Idiopat thrombocytop pur, Idiopath thrombocytopen purpur, Immune thrombocytopenic purpur, IDIOPATHIC THROMBOCYTOPENIC PURPURA, ITP, Idiopathic thrombocytopenic purpura (ITP), THROMBOCYTOPENIC PURPURA, AUTOIMMUNE, WERLHOF DIS, WERLHOFS DIS, immune thrombocytopenic purpura (diagnosis), immune thrombocytopenic purpura, idiopathic thrombocytopenic purpura (diagnosis), ITP (idiopathic thrombocytopenic purpura), idiopathic thrombocytopenic purpura, idiopathic thrombocytopenic purpura (ITP), ITP (immune thrombocytopenic purpura), Idiopathic Thrombocytopenia Purpura, Idiopathic Thrombocytopenia, IMMUNE THROMBOCYTOPENIC PURPURA, AITP, Immune thrombocytopenic purpura, Immune thrombocyt purpra, Purpura, Thrombocytopenic, Idiopathic [Disease/Finding], idiopathic purpura, immune purpura thrombocytopenic, thrombocytopenic purpura immune, werlhof's disease, Immune Thrombocytopenic Purpura, Thrombocytopenic Purpura, Autoimmune, Idiopathic thrombocytopenia purpura, Idiopathic purpura (& thrombocytopenic), Ideopath thrombocytopenic pur, ITP - idiopathic thrombocytopenic purpura, Idiopathic purpura (& thrombocytopenic) (disorder), ITP - Idiopathic thrombocytopenic purpura, Werlhof's syndrome, Immune Thrombocytopenias, Autoimmune Thrombocytopenia, Purpuras, Immune Thrombocytopenic, Thrombocytopenic Purpuras, Immune, Thrombocytopenia, Immune, Purpura, Immune Thrombocytopenic, Thrombocytopenias, Immune, Thrombocytopenias, Autoimmune, Thrombocytopenic Purpura, Immune, Immune Thrombocytopenia, Thrombocytopenia, Autoimmune, Immune Thrombocytopenic Purpuras, Autoimmune Thrombocytopenias, Idiopathic thrombocytopenic purpura, Werlhof's disease, Autoimmune thrombocytopenic purpura, Idiopathic purpura, Idiopathic thrombocytopenic purpura (disorder), Immune thrombocytopenic purpura (disorder), Frank, Werlhof, idiopathic; purpura, purpura; idiopathic, purpura; thrombocytopenic, idiopathic, thrombocytopenic; purpura, idiopathic, ITP, NOS, Idiopathic thrombocytopenic purpura, NOS, Idiopathic purpura, NOS, Idiopath Thrombocytopenic Purp
Dutch idiopathische purpura, syndroom van Werlhof, ITP, immune trombocytopenische purpura, idiopathische trombocytopenische purpura, idiopathisch; purpura, purpura; idiopathisch, purpura; trombocytopenisch, idiopathisch, trombocytopenisch; purpura, idiopathisch, Auto-immune trombocytopenische purpura, Idiopathische trombocytopenische purpura, Idiopathische trombocytopenische purpura (ITP), Purpura, idiopathische trombocytopenische (ITP), Purpura, trombocytopenische auto-immune, Trombocytopenische purpura (ITP), idiopathische, Werlhof, ziekte van
French Purpura idiopathique, PTI (Purpura Thrombocytopénique Idiopathique), Purpura thrombopénique idiopathique de Werlhof, PTI, Purpura thrombopénique immun, Syndrome de Werlhof, Purpura thrombopénique idiopathique, Maladie de Werlhof, PTAI, Purpura thrombocytopénique auto-immun, Purpura thrombocytopénique autoimmun, Purpura thrombocytopénique idiopathique, Purpura thrombopénique auto-immun, Purpura thrombopénique autoimmun
German idiopathische Purpura, Purpura idiopathisch thrombozytopenisch, Immunthrombozytopenische Purpura, Werlhofsyndrom, ITP, Autoimmun-thrombozytopenische Purpura, Idiopathische thrombozytopenische Purpura, Purpura, thrombozytopenische, Autimmun-, Purpura, thrombozytopenische, idiopathische, Werlhof-Syndrom
Italian Porpora idiopatica, Porpora trombocitopenica autoimmune, Morbo di Werlhof, Porpora trombocitopenica immune, Sindrome di Werlhof, Porpora trombocitopenica idiopatica
Portuguese Púrpura idiopática, Púrpura Trombocitopênica Autoimune, Púrpura trombocitopénica idiopática, Púrpura trombocitopénica imune, Síndrome de Werlhof, Doença de Werlhof, Púrpura Trombocitopênica Idiopática
Spanish Púrpura idiopática, púrpura trombocitopénica autoinmunitaria, púrpura trombocitopénica autoinmune (trastorno), púrpura trombocitopénica autoinmune, púrpura trombocitopénica autoinmunitaria (trastorno), Púrpura trombocitopénica inmune, Síndrome de Werlhof, Púrpura trombocitopénica idiopática, PTI, enfermedad de Werlhof, púrpura idiopática, púrpura trombocitopénica idiopática (trastorno), púrpura trombocitopénica idiopática, Enfermedad de Werlhof, Púrpura Trombocitopénica Idiopática, Púrpura Trombocitopénica Autoinmune
Japanese 特発性紫斑病, トクハツセイシハンビョウ, 紫斑病-特発性血小板減少性, ウェルホーフ紫斑, トクハツセイケッショウバンゲンショウセイシハンビョウ, 免疫性血小板減少性紫斑病, ITP, ウェルホーフ症候群, メンエキセイケッショウバンゲンショウセイシハンビョウ, ITP, ウェルホーフショウコウグン, ウェルホーフ病, ウェルホーフ紫斑病, 特発性血小板減少性紫斑, 特発性血小板減少性紫斑病, 特発血小板減少性紫斑病, 自己免疫性血小板減少性紫斑病, ウェルルホフ病, ヴェルホーフ病, ヴェルルホフ病, 紫斑病-自己免疫性血小板減少性, 紫斑病-血小板減少性-特発性, 紫斑病-血小板減少性-自己免疫性, 血小板減少性紫斑病-特発性
Swedish Purpura, trombocytopen, idiopatisk
Czech autoimunitní trombocytopenická purpura, purpura trombocytopenická idiopatická, Werlhofova nemoc, Idiopatická purpura, idiopatická trombocytopenická purpura, Idiopatická trombocytopenická purpura, ITP, Werlhofův syndrom, Imunitní trombocytopenická purpura
Finnish Idiopaattinen trombosytopeeninen purppura
Russian IDIOPATICHESKAIA TROMBOTSITOPENICHESKAIA PURPURA, AUTOIMMUNNAIA TROMBOTSITOPENICHESKAIA PURPURA, PURPURA TROMBOTSITOPENICHESKAIA IDIOPATICHESKAIA, PURPURA TROMBOTSITOPENICHESKAIA AUTOIMMUNNAIA, VERL'GOFA BOLEZN', АУТОИММУННАЯ ТРОМБОЦИТОПЕНИЧЕСКАЯ ПУРПУРА, ВЕРЛЬГОФА БОЛЕЗНЬ, ИДИОПАТИЧЕСКАЯ ТРОМБОЦИТОПЕНИЧЕСКАЯ ПУРПУРА, ПУРПУРА ТРОМБОЦИТОПЕНИЧЕСКАЯ АУТОИММУННАЯ, ПУРПУРА ТРОМБОЦИТОПЕНИЧЕСКАЯ ИДИОПАТИЧЕСКАЯ
Korean 특발성 혈소판 감소성 자색반증
Croatian PURPURA, IDIOPATSKA TROMBOCITOPENIČNA
Polish Immunotrombocytopenia samoistna, Małopłytkowość przewlekła, Plamica małopłytkowa idiopatyczna, Małopłytkowość samoistna, Choroba Werlhofa, Choroba Franka, Plamica małopłytkowa samoistna, Plamica małopłytkowa autoimmunologiczna, Małopłytkowość ostra
Hungarian Idiopathiás purpura, Immun thrombocytopeniás purpura, Werlhof-syndroma, ITP, idiopathiás thrombocytopeniás purpura
Norwegian Idiopatisk trombocytopenisk purpura, Purpura thrombocytopenica idiopathica, Autoimmun trombocytopenisk purpura