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Essential Thrombocytemia
Dimas Bayu
Tutik Harjianti
A. Fachruddin
Division of Hematology & Oncology - Dept. of Internal Medicine
Faculty of Medicine, Hasanuddin University
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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.
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Division of Hematology & Oncology Dept. of Internal Medicine
Essential Thrombocytemia (ET)
Myeloproliferative disorders
Idiopathic
megakaryocyte proliferation in bonemarrow>> thrombocyte>>
Essential
Thrombocytemia
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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!.
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Division of Hematology & Oncology Dept. of Internal Medicine
Sign & Symptom
ET
B
E
C
D
A50-60 years old
Thrombocyte >>
Initial sign:Thrombosis
Venous thrombosis:hepatic, portal & mesenteric
Pain & erythemaof the hands
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Division of Hematology & Oncology Dept. of Internal Medicine
Sign & Symptom
Bleeding:mostly occurred in mucosal
as a manifestation of thrombocyte defect
Splenomegaly: occurred in 25% patients
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Division of Hematology & Oncology Dept. of Internal Medicine
Management
Hydroxy-Urea0,5 2,0 g / daySide effect: neutropenia
Anagrelide2 4 mg / daySide effect: cephalgia, anemia,
periferal edema, congestive heart failure
Acetyl Salicylic Acid (ASA)to reduce vasomotor symptom
(eritromelalgia & parestesi)
Tx
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Division of Hematology & Oncology Dept. of Internal Medicine
Management
Risk forthrombosis
will be high if
PLT > 500.000/mm3
Severe bleedingcould
be reduce by using
plateletpheresis
(equipment to discard
excessive blood)
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Complication:
!Very high platelet counts are associated primarily with
hemorrhage.
!
Platelet counts
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Division of Hematology & Oncology Dept. of Internal Medicine
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 betransformed into acute leukemia
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