II. Epidemiology

  1. More common in women

III. Symptoms and signs

  1. Common
    1. Headache
    2. Paresthesias
  2. Less common
    1. Thrombotic events
    2. Bleeding events

IV. Differential Diagnosis

  1. Reactive Thrombocytosis
  2. Clonal Thrombocytosis
  3. Chronic myeloproliferative disorder

V. Labs (rule-out differential diagnosis above)

VI. Management

  1. Age Under 60 years
    1. Low risk patients
      1. Consider low dose Aspirin
    2. Intermediate risk patients
      1. Platelet Count <1500 x10^3/uL
        1. Anagrelide
        2. Low dose Aspirin
      2. Platelet Count >1500 x10^3/uL
        1. Anagrelide
    3. High risk patients
      1. Hydroxyurea
      2. Anagrelide
      3. Low dose Aspirin
  2. High risk patients over age 60 years
    1. Low dose Aspirin
    2. Hydroxyurea
  3. Women of child bearing age
    1. Consider low dose Aspirin
    2. Interferon alfa in high risk patients

VII. Complications (low Incidence)

VIII. Prognosis: Factors predictive of poor prognosis

  1. Age over 60 years
  2. Thrombosis history

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Ontology: Thrombocythemia, Essential (C0040028)

Definition (MSHCZE) Patologický stav s dlouhodobě zvýšeným počtem trombocytů v krvi v důsledku jejich nadměrné tvorby v kostní dřeni. V kostní dřeni je hyperplazie megakaryocytární řady, v periferní krvi je výrazně zvýšen počet destiček, ty však jsou funkčně defektní. Proto kromě trombotických příhod mohou být i krvácivé stavy, v popředí fyzikálního nálezu je splenomegalie. Léčí se některými cytostatiky. Přechodné reaktivní zvýšení počtu destiček se nazývá trombocytóza. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. It is characterized by sustained thrombocytosis in the blood, increased numbers of large, mature megakaryocytes in the bone marrow, and episodes of thrombosis and/or hemorrhage. The cause is unknown. Median survival times of 10-15 years are commonly reported. (WHO, 2008)
Definition (NCI_NCI-GLOSS) An increased number of thrombocytes (platelets) in the blood, without a known cause.
Definition (MSH) A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
Definition (CSP) clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
Concepts Disease or Syndrome (T047)
MSH D013920
ICD9 238.71
ICD10 M9962/1 , D47.3, D75.2
SnomedCT 109994006, 65471002, 189508006, 307652003, 191333009, 234499005, 189513005, 189514004, 128844009
English Essential Thrombocythemias, Hemorrhagic Thrombocythemia, Hemorrhagic Thrombocythemias, Idiopathic Thrombocythemias, Primary Thrombocythemias, Thrombocythemia, Idiopathic, Thrombocythemia, Primary, Thrombocythemias, Essential, Thrombocythemias, Hemorrhagic, Thrombocythemias, Idiopathic, Thrombocythemias, Primary, hemorrhagic thrombocythemia, ET - Essent thrombocythaemia, ET - Essent thrombocythemia, ET - Essential thrombocythaemia, ET - Essential thrombocythemia, Essent (haemorrh) thrombocyth, Essential (haemorrhagic) thrombocythaemia, Essential (hemorrhagic) thrombocythemia, Idiopathic (hemorrhagic) thrombocythemia, Primary thrombocytosis, THROMBOCYTHEMIA IDIOPATHIC, primary thrombocythemia, [M]Idiopathic thrombocythaemia, [M]Idiopathic thrombocythemia, Essential thrombocythaemia (clinical disorder), Essential thrombocythemia (clinical), essential thrombocytosis (diagnosis), idiopathic thrombocythemia, essential thrombocythemia (diagnosis), essential thrombocythemia, primary thrombocytosis, primary thrombocytosis (diagnosis), idiopathic thrombocythemia (diagnosis), essential thrombocytosis, Idiopathic thrombocythaemia -RETIRED-, essential hemorrhagic thrombocythemia (diagnosis), essential hemorrhagic thrombocythemia, Thrombocythemia, Essential, Thrombocythemia, Hemorrhagic, Ideopathic thrombocytosis, Essntial thrombocythemia, Thrombocythemia, Essential [Disease/Finding], thrombocythemia essential, Thrombocytosis;essential, essential thrombocythaemia, thrombocytosis essential, Thrombocytoses, Primary, Thrombocytosis, Primary, Primary Thrombocytoses, Primary Thrombocythemia, Essential thrombocytosis (disorder), Idiopathic thrombocythemia (disorder), Idiopathic thrombocytosis, Idiopathic thrombocythaemia (disorder), Idiopathic thrombocythemia (morphologic abnormality), Idiopathic thrombocythemia, Essential hemorrhagic thrombocythemia, Essential thrombocythaemia, Essential thrombocythemia, Idiopathic hemorrhagic thrombocythemia, Essential haemorrhagic thrombocythaemia, Idiopathic haemorrhagic thrombocythaemia, Idiopathic thrombocythaemia, Essential thrombocythemia (disorder), Essential thrombocythemia (morphologic abnormality), thrombocythemia, essential, thrombocytosis; essential, Essential thrombocythaemia (clinical), Essential thrombocythaemia (disorder), Idiopathic thrombocythemia -RETIRED-, Essential Thrombocythemia, Idiopathic Thrombocythemia, Essential Thrombocytemia, Essential Thrombocytosis, Primary Thrombocytosis, Essential thrombocytosis, Essential thrombocythemia (clinical disorder)
Dutch essentiële thrombocythemia, essentiële thrombocytosis, idiopathische trombocytose, essentiële trombocytemie, trombocytose; essentieel, Essentiële (hemorragische) trombocytemie, Essentiële trombocytose, Hemorragische trombocytemie, Trombocytemie, hemorragische, Trombocytemie, idopathische, Trombocytemie, primaire, Essentiële hemorragische trombocytemie, Trombocytemie, essentiële
French Thrombocythémie essentielle, Thrombocytose essentielle, Thrombocytose idiopathique, Thrombocytémie essentielle, Thrombocytémie hémorragique, Thrombocytémie primitive, Thrombocytose primitive, Hyperplaquettose primitive
German ideopathische Thrombozytose, idiopathische Thrombozytose, essentielle Thrombozytose, Essentielle (haemorrhagische) Thrombozythaemie, Essentielle Thrombozytose, essentielle Thrombozythaemie, Hämorrhagische Throbozytämie, Thrombozytämie, essentielle, Thrombozytämie, hämorrhagische, Thrombozytämie, idiopathische, Thrombozytämie, primäre
Italian Trombocitosi idiopatica, Trombocitemia essenziale, Trombocitemia idiopatica, Trombocitemia emorragica, Trombocitemia primaria
Portuguese Trombocitose essencial, Trombocitose idiopática, Trombocitemia Hemorrágica, Trombocitemia Essencial, Trombocitemia essencial, Trombocitemia Idiopática, Trombocitemia Primária
Spanish Trombocitosis idiopática, Trombocitosis esencial, trombocitemia esencial (clínico), trombocitemia hemorrágica idiopática (clínica), [M] trombocitemia idiopática, Trombocitemia Esencial, Trombocitemia Hemorrágica, trombocitemia idiopática (trastorno), trombocitosis esencial (trastorno), trombocitosis esencial, trombocitemia hemorrágica esencial, trombocitosis hemorrágica idiopática, trombocitemia idiopática (concepto no activo), trombocitemia esencial (anomalía morfológica), trombocitemia esencial (trastorno), trombocitemia esencial hemorrágica, trombocitemia esencial, trombocitemia idiopática hemorrágica, trombocitemia idiopática, Trombocitemia esencial, Leucemia Idiopática, Trombocitemia Primaria
Japanese 特発性血小板増加症, 本態性血小板増加症, ホンタイセイケッショウバンケツショウ, ホンタイセイケッショウバンゾウカショウ, ホンタイセイケッショウバンケッショウ, トクハツセイケッショウバンゾウカショウ, 血小板血症-原発性, 血小板血症-出血性, 出血性血小板血病, 出血性血小板血症, 原発性栓球血症, 本態性栓球血症, 特発性栓球血症, 原発性血小板血症, 本態性血小板血症, 特発性血小板血症, 血小板血症-本態性, 血小板血症-特発性
Finnish Essentiaalinen trombosytemia
Russian LEIKEMIIA MEGAKARIOTSITARNAIA, GEMORRAGICHESKAIA TROMBOTSITEMIIA, TROMBOTSITEMIIA IDIOPATICHESKAIA, TROMBOTSITEMIIA ESSENTSIAL'NAIA, TROMBOTSITEMIIA PERVICHNAIA, TROMBOTSITEMIIA GEMORRAGICHESKAIA, ТРОМБОЦИТЕМИЯ ЭССЕНЦИАЛЬНАЯ, ТРОМБОЦИТЕМИЯ ГЕМОРРАГИЧЕСКАЯ, ГЕМОРРАГИЧЕСКАЯ ТРОМБОЦИТЕМИЯ, ЛЕЙКЕМИЯ МЕГАКАРИОЦИТАРНАЯ, ТРОМБОЦИТЕМИЯ ИДИОПАТИЧЕСКАЯ, ТРОМБОЦИТЕМИЯ ПЕРВИЧНАЯ
Czech Idiopatická trombocytóza, Esenciální trombocytemie, Esenciální trombocytóza, trombocytémie hemoragická, trombocytémie idiopatická, trombocytémie primární, esenciální trombocytemie, trombocytémie esenciální, primární trombocytemie
Korean 본태성(출혈성) 혈소판 증가증, 본태성 혈소판증가증
Swedish Trombocytemi, essentiell
Polish Trombocytopenia krwotoczna, Trombocytopenia idiopatyczna, Nadpłytkowość krwotoczna, Nadpłytkowość pierwotna, Nadpłytkowość samoistna, Nadpłytkowość idiopatyczna
Hungarian Essentialis thrombocythaemia, Idiopathias thrombocytosis, Idiopathiás thrombocytosis, Essentialis thrombocytosis
Norwegian Essensiell trombocytemi, Essensiell hemoragisk trombocytemi, Idiopatisk hemoragisk trombocytemi