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.

 Trombositosis Esensial:
Kelainan klonal sel induk hematopoetik multipotensial, termasuk kelainan mieloproliferatif dengan ekspresi fenotipe predominan pada jalur megakariosit dan trombosit. TE: Kadar trombopoeti normal/meingkat meskipun terjadi peningkatan massa trombosis dan megakariosit. Disregulasi kadar trombopoetin plasma pd TE diduga disebabkan: - Produksi trombopoeti yg berlebihan dan/atau - Abnormalitas pengikatan dan pemakaian trombopoetin oleh trombosit dan megakaryosit
Division of Hematology & Oncology Dept. of Internal Medicine

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: 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

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

Mekanisme terjadinya trombosis dan perdarahan pada TE diduga:

Trombosis karena: - Peningkatan massa trombosit disertai hiperagregabilitas trombosit - Aktivitas hemostasis oleh lekosit polimorfonuklear.
Perdarahan karena: - Abnormalitas fungsi trombosit (defek kwalitatif) atau inhibisi koagulasi karena trombosis
Division of Hematology & Oncology Dept. of Internal Medicine

Kriteria Diagnosis TE
I. Jumlah trombosit >600.000/mm3 II. Hematokrit <0.46 atau massa eritrosit normal III. Cadangan Fe sumsum tulang normal (dengan pewarnaan) atau serum feritin normal atau MCV normal IV. Tidak didapatkan kromosom Philadelphia atau mutasi bcr/abl V. Fibrosis kolagen pada sumsum tulang tidak ada atau kurang 1/3 area biopsi, tanpa disertai splenomegali yang menonjol dan reaksi lekoeritroblastik VI. Tidak didapatkan kelainan morfologi atau sitogenetik sindroma mielodisplasia VII. Tidak didapatkan penyebab reaktif trombositosis
Int J Hematology,2002;76:311-7

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

Acetyl Salicylic Acid (ASA) to reduce vasomotor symptom (eritromelalgia & parestesi)

Hydroxy-Urea 0,5 2,0 g / day Side effect: neutropenia

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