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

Essential
Idiopathic Thrombocytemia

megakaryocyte proliferation in bone

marrow >> thrombocyte >>

Division of Hematology & Oncology Dept. of Internal Medicine

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 A C Initial sign:

Thrombosis

ET

Venous thrombosis: E D Pain & erythema

hepatic, portal & mesenteric 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)

II. Inflammation (RA, colitis) & chronic infection

DD III. PV (hematocryte & RBC mass >>)

Division of Hematology & Oncology Dept. of Internal Medicine

 Management

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

Acetyl Salicylic Acid (ASA) Side effect: neutropenia
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

Severe bleeding could

Risk for thrombosis be reduce by using
will be high if plateletpheresis
PLT > 500.000/mm3 (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 !