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Essential Thrombocytosis

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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

Essential thrombocytosis:
vIs a clonal disorder of unknown etiology.
vOver production of platelets in the absence of
definable reason.
vIt can occur at any age group.
vUnexplained female predominance.

Essential Thrombocytemia (ET)

Myeloproliferative disorders
Essential
Thrombocytemia

Idiopathic
megakaryocyte proliferation in bone
marrow >> thrombocyte >>

Division of Hematology & Oncology Dept. of Internal Medicine

Clinical feature &diagnosis:


Symptoms:
vNo specific symptoms .
vThese patients have hemorrhagic& thrombotic tendency.
(easy bruising,microvascular occlusion,).
Physical examination :
vusually unremarkable except for the presence of mild
splenomegally.
Lab investigation:
vAnemia is unusual.Mild leukocytosis.
vBlood smear : most remarkable for the number of PLT.
vLeukocyte alkaline phosphatase is either normal or.

Sign & Symptom


Thrombocyte >>

50-60 years old

ET
Venous thrombosis:
hepatic, portal & mesenteric

Initial sign:
Thrombosis

Pain & erythema


of the hands

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

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:

vVery high platelet counts are associated primarily with


hemorrhage.
vPlatelet counts <a million are more often associated with
thrombosis.
vFrom 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

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