Hematology and Oncology Book

http://www.fpnotebook.com/

Essential Thrombocythemia

Advertisement

  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, Hemorrhagic (C0040028)

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.
Definition (NCI)A chronic myeloproliferative disorder that involves primarily the megakaryocytic lineage and 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, 2001) -- 2003
ConceptsDisease or Syndrome (T047)
ICD9238.71, 238.71
MSHD013920
EnglishEssential haemorrhagic thrombocythaemia, Essential hemorrhagic thrombocythemia, Essential Thrombocytemia, Essential thrombocythaemia, Essential Thrombocythemia, Essential Thrombocythemias, Essential thrombocytosis, ESSNTIAL THROMBOCYTHEMIA, ET - Essential thrombocythaemia, ET - Essential thrombocythemia, Hemorrhagic Thrombocythemia, Hemorrhagic Thrombocythemias, Idiopathic haemorrhagic thrombocythaemia, Idiopathic hemorrhagic thrombocythemia, Idiopathic thrombocythaemia, Idiopathic thrombocythaemia -RETIRED-, Idiopathic Thrombocythemia, Idiopathic thrombocythemia -RETIRED-, Idiopathic Thrombocythemias, Primary Thrombocythemia, Primary Thrombocythemias, Primary thrombocytosis, THROMBOCYTHEMIA IDIOPATHIC
Spanishtrombocitemia esencial, trombocitemia esencial hemorragica, trombocitemia hemorragica esencial, trombocitemia idiopatica, trombocitemia idiopatica hemorragica, trombocitosis esencial, trombocitosis hemorragica idiopatica
Parent ConceptsBlood Platelet Disorders (C0005818), Neoplasm of uncertain behavior of other lymphatic and hematopoietic tissue (C0027632), Blood Coagulation Disorders (C0005779), Hemorrhagic Disorders (C0019087), thrombocytosis (C0836924), Chronic myeloproliferative disorder (C1292778), Megakaryocytic Neoplasm (C1334687), stage, chronic myeloproliferative disorders (C0279215), [X]Malignant neoplasms of lymphoid, hematopoietic and related tissue (C0348393), Duplicate concept (C1274013), Reason not stated concept (C1276325), Primary malignant neoplasm (C1306459), Disorder of hematopoietic morphology (C1532234)
SourcesCOSTAR, CSP, DXP, ICD9CM, MSH, MTHICD9, NCI, NDFRT, OMIM, PDQ, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



Navigation Tree