Hematology and Oncology Book

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Essential Thrombocythemia

Aka: Essential Thrombocythemia
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  1. See Also
    1. Thrombocytosis
  2. Epidemiology
    1. More common in women
  3. Symptoms and signs
    1. Common
      1. Headache
      2. Paresthesias
    2. Less common
      1. Thrombotic events
      2. Bleeding events
  4. Differential Diagnosis
    1. Reactive Thrombocytosis
    2. Clonal Thrombocytosis
    3. Chronic myeloproliferative disorder
  5. Labs (rule-out differential diagnosis above)
    1. C-Reactive Protein (CRP) Normal
    2. Erythrocyte Sedimentation Rate Normal
    3. Fibrinogen Level Normal
    4. Bone Marrow Biopsy Normal
  6. 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
  7. Complications (low Incidence)
    1. Acute Leukemia
    2. Myelofibrosis
  8. Prognosis: Factors predictive of poor prognosis
    1. Age over 60 years
    2. Thrombosis history
  9. References
    1. Tefferi (2001) Am J Med 109:146

Thrombocythemia, Essential (C0040028)

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 (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.
Definition (NCI) An increased number of thrombocytes (platelets) in the blood, without a known cause.
Concepts Disease or Syndrome (T047)
MSH D013920
ICD9 238.71
ICD10 M9962/1, D47.3, D75.2
SnomedCT 109994006, 65471002, 189513005, 189514004, 234499005, 128844009, 189508006, 307652003, 191333009
English Essential Thrombocythemias, Hemorrhagic Thrombocythemia, Hemorrhagic Thrombocythemias, Idiopathic Thrombocythemias, Primary Thrombocythemias, Thrombocythemia, Idiopathic, Thrombocythemia, Primary, Thrombocythemias, Essential, Thrombocythemias, Hemorrhagic, Thrombocythemias, Idiopathic, Thrombocythemias, Primary, ESSENTIAL THROMBOCYTHEMIA, THROMBOCYTHEMIA, ESSENTIAL, WITH HEMORRHAGIC DIATHESIS, THROMBOCYTHEMIA, HEMORRHAGIC, THROMBOCYTHEMIA, IDIOPATHIC, THROMBOCYTHEMIA, PRIMARY HEMORRHAGIC, 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, THROMBOCYTHEMIA, ESSENTIAL, THROMBOCYTOSIS, PRIMARY, THROMBOCYTOSIS, AUTOSOMAL DOMINANT, primary 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-, Idiopathic thrombocythemia (morphologic abnormality), Idiopathic thrombocythaemia (disorder), essential hemorrhagic thrombocythemia (diagnosis), essential hemorrhagic thrombocythemia, Thrombocythemia, Essential, Thrombocythemia, Hemorrhagic, Ideopathic thrombocytosis, THROMBOCYTOSIS ESSENTIAL, Essntial thrombocythemia, Thrombocythemia, Essential [Disease/Finding], thrombocythemia essential, Thrombocytosis;essential, essential thrombocythaemia, thrombocytosis essential, Idiopathic thrombocytosis, 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-, Primary Thrombocythemia, Essential Thrombocythemia, Idiopathic Thrombocythemia, Essential Thrombocytemia, Essential Thrombocytosis, Primary Thrombocytosis, Essential thrombocytosis, [M]Idiopathic thrombocythaemia, [M]Idiopathic thrombocythemia, Essential thrombocythemia (clinical disorder), Essential thrombocytosis (disorder), Idiopathic thrombocythemia (disorder)
Dutch essentiële thrombocythemia, essentiële thrombocytosis, idiopathische trombocytose, trombocytose; essentieel, Essentiële (hemorragische) trombocytemie, Essentiële trombocytose, essentiële thrombocytemie, 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 idiopathique
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), trombocitemia idiopática (trastorno), [M] trombocitemia idiopática, trombocitosis esencial (trastorno), Essential thrombocythaemia, Idiopathic thrombocythemia, ET - Essential thrombocythemia, Essential (hemorrhagic) thrombocythemia, Idiopathic thrombocythaemia, Essential (haemorrhagic) thrombocythaemia, ET - Essential thrombocythaemia, Essential thrombocythemia, Trombocitemia Esencial, Trombocitemia Hemorrágica, Trombocitemia Hemorragica, trombocitemia esencial (anomalía morfológica), trombocitemia esencial (trastorno), trombocitemia esencial hemorrágica, trombocitemia esencial, trombocitemia hemorrágica esencial, trombocitemia idiopática (concepto no activo), trombocitemia idiopática hemorrágica, trombocitemia idiopática, trombocitosis esencial, trombocitosis hemorrágica idiopática, Trombocitemia esencial, Leucemia Idiopática, Trombocitemia Primaria, Leucemia Idiopatica
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ﾊﾞﾝｹﾂｼｮｳ, ﾎﾝﾀｲｾｲｹｯｼｮｳﾊﾞﾝｿﾞｳｶｼｮｳ, ﾎﾝﾀｲｾｲｹｯｼｮｳﾊﾞﾝｹｯｼｮｳ, ﾄｸﾊﾂｾｲｹｯｼｮｳﾊﾞﾝｿﾞｳｶｼｮｳ, 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 esenciální, trombocytémie hemoragická, trombocytémie idiopatická, trombocytémie primární
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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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