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    Essential Thrombocytemia: Rahmawati Minhajat A. Fachruddin Benyamin

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    HajrahPalembangan
    Essential thrombocytosis, also known as essential thrombocythemia (ET), is a clonal myeloproliferative disorder characterized by overproduction of platelets in the bone marrow in the absence of an identifiable cause. ET can occur at any age but predominantly affects women ages 50-60 years old. Patients present with hemorrhagic and thrombotic symptoms such as easy bruising, microvascular occlusion, venous thrombosis in the liver, portal veins, and mesentery. Diagnosis involves blood tests showing elevated platelet counts but normal or elevated white blood cell and leukocyte alkaline phosphatase levels. Treatment focuses on reducing platelet counts and thrombosis risk using hydroxyurea, anagrelide, or aspirin.

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    Essential Thrombocytemia: Rahmawati Minhajat A. Fachruddin Benyamin

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    HajrahPalembangan
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    Essential thrombocytosis, also known as essential thrombocythemia (ET), is a clonal myeloproliferative disorder characterized by overproduction of platelets in the bone marrow in the absence of an identifiable cause. ET can occur at any age but predominantly affects women ages 50-60 years old. Patients present with hemorrhagic and thrombotic symptoms such as easy bruising, microvascular occlusion, venous thrombosis in the liver, portal veins, and mesentery. Diagnosis involves blood tests showing elevated platelet counts but normal or elevated white blood cell and leukocyte alkaline phosphatase levels. Treatment focuses on reducing platelet counts and thrombosis risk using hydroxyurea, anagrelide, or aspirin.

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    ET(new)

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    Essential thrombocytosis, also known as essential thrombocythemia (ET), is a clonal myeloproliferative disorder characterized by overproduction of platelets in the bone marrow in the absence of an identifiable cause. ET can occur at any age but predominantly affects women ages 50-60 years old. Patients present with hemorrhagic and thrombotic symptoms such as easy bruising, microvascular occlusion, venous thrombosis in the liver, portal veins, and mesentery. Diagnosis involves blood tests showing elevated platelet counts but normal or elevated white blood cell and leukocyte alkaline phosphatase levels. Treatment focuses on reducing platelet counts and thrombosis risk using hydroxyurea, anagrelide, or aspirin.

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    Download as ppsx, pdf, or txt
    23 views12 pages

    Essential Thrombocytemia: Rahmawati Minhajat A. Fachruddin Benyamin

    Uploaded by

    HajrahPalembangan
    Essential thrombocytosis, also known as essential thrombocythemia (ET), is a clonal myeloproliferative disorder characterized by overproduction of platelets in the bone marrow in the absence of an identifiable cause. ET can occur at any age but predominantly affects women ages 50-60 years old. Patients present with hemorrhagic and thrombotic symptoms such as easy bruising, microvascular occlusion, venous thrombosis in the liver, portal veins, and mesentery. Diagnosis involves blood tests showing elevated platelet counts but normal or elevated white blood cell and leukocyte alkaline phosphatase levels. Treatment focuses on reducing platelet counts and thrombosis risk using hydroxyurea, anagrelide, or aspirin.

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    of 12

    Essential Thrombocytemia

    Rahmawati Minhajat
    A. Fachruddin Benyamin
    Division of Hematology & Oncology - Dept. of Internal Medicine
    Faculty of Medicine, Hasanuddin University

    Essential thrombocytosis:
    Is a clonal disorder of unknown etiology.
    Over production of platelets in the absence of
    definable reason.
    It can occur at any age group.
    Unexplained female predominance.

    Essential Thrombocytemia (ET)

    Myeloproliferative disorders

    Idiopathic
    megakaryocyte proliferation in bone
    marrow >> thrombocyte >>

    Division of Hematology & Oncology Dept. of Internal Medicine

    Essential
    Thrombocytemia

    Clinical feature &diagnosis:


    Symptoms:
    No specific symptoms .
    These patients have hemorrhagic& thrombotic tendency.
    (easy bruising,microvascular occlusion,).
    Physical examination :
    usually unremarkable except for the presence of mild
    splenomegally.
    Lab investigation:
    Anemia is unusual.Mild leukocytosis.
    Blood smear : most remarkable for the number of PLT.
    Leukocyte alkaline phosphatase is either normal or.

    Sign & Symptom


    Thrombocyte >>

    50-60 years old

    ET
    Venous thrombosis:
    hepatic, portal & mesenteric

    Division of Hematology & Oncology Dept. of Internal Medicine

    Initial sign:
    Thrombosis

    Pain & erythema


    of the hands

    Sign & Symptom

    Bleeding: mostly occurred in mucosal


    as a manifestation of thrombocyte defect

    Splenomegaly: occurred in 25% patients

    Division of Hematology & Oncology Dept. of Internal Medicine

    Differential Diagnosis

    I. Reactive thrombocytosis (rarely >1.000.000/ mm3)

    DD

    II. Inflammation (RA, colitis) & chronic infection


    III. PV (hematocryte & RBC mass >>)

    Division of Hematology & Oncology Dept. of Internal Medicine

    Management

    Hydroxy-Urea 0,5 2,0 g / day


    Side effect: neutropenia

    Acetyl Salicylic Acid (ASA)


    to reduce vasomotor symptom
    (eritromelalgia & parestesi)

    Tx

    Anagrelide 2 4 mg / day
    Side effect: cephalgia, anemia,
    periferal edema, congestive heart failure
    Division of Hematology & Oncology Dept. of Internal Medicine

    Management

    Risk for thrombosis


    will be high if
    PLT > 500.000/mm3

    Severe bleeding could


    be reduce by using
    plateletpheresis
    (equipment to discard
    excessive blood)

    Division of Hematology & Oncology Dept. of Internal Medicine

    Complication:

    Very high platelet counts are associated primarily with


    hemorrhage.
    Platelet counts <a million are more often associated with
    thrombosis.
    From the neurological problems migraine-related is the
    commonest(respond to platelet lowering).,Erythromelalgia
    (respond to cyclooxygenase inhibitors).

    Prognosis

    ET: Indolent
    Long-term survival
    Developed country: 15 years after first
    diagnosis
    Major problem: thrombosis
    Late phase: Bone marrow fibrosis,
    splenomegaly / spleen infarction, 5% will be
    transformed into acute leukemia

    Division of Hematology & Oncology Dept. of Internal Medicine

    Thank You !

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