Thrombocytosis uniba 16-1-13

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Transcript of Thrombocytosis uniba 16-1-13

Thrombocytosisdr Putra Hendra SpPDUNIBA

PlateletGranule :Glycoprotein Thrombospondin VEGF PDGF

Angiogenesis

Dense granule :Ca Serotonin ADP Thromboxane A2 Aggregation

Platelet aggregation

Definition of Thrombocytosis

Platelet count > 450,000/ml

Three Major Causes .. Familial thrombocytosis Clonal thrombocytosis Reactive thrombocytosis

Familial ThrombocytosisRare case reports Hereditary mutation of TPO gene mutation of c-mpl receptor Overproduction of thrombopoitein

Clonal Thrombocytosis Essential thrombocythemia Polycythemia Vera Chronic Myelocytic Leukemia Myelofibrosis with Myeloid Metaplasia

Reactive Thrombocytosis Infection Malignancy Autoimmune diseases Postsplenectomy Trauma Rebound thrombocytosis Anemia Hemorrhage Drug(Vincristine,steroid), etc

How to differentiate ?Clonalmyeloproliferative dysregulation malignant changes thrombotic/hemo-rrhagic complication

Reactivesecondary responses physiological feedback transient and subsides exceedingly rare/ Kawasaki disease

Clonalmpl

ReactiveP PTPO IL-6 TPO IL-3 IL-11 GM-CSF EPO

Sensitivity to TPO

Mega

Mega

Proliferation

Proliferation

DiagnosisClonal thrombocythemia Blood SmearMyelocyte Metamyelocyte Thrombocyte

Bone Marrow aspirate/biopsyGiant megakaryocyte Hypercellularity Hyperplasia

Diagnosis Clonal thrombocythemia Cytogenetic studyCML,Ph1,bcr-abl

Platelet aggregation testADP,Epinephrine

ET - Symptoms Many patients are asymptomatic at time of diagnosis Digital ischemia from microvascular thrombi Erythromelalgia Pruritis Hemorrhage - seen in 40% of pts

ComplicationClonal thrombocytemiaNeurologic:Headache TIA

Thrombosis:Coronary Renal Portal DVT, Pul

Bleeding:GI Skin Eye Brain Urinary tract

ComplicationClonal thrombocytemiaPregnancy:Spontaneous abortion IUGR

Others:Erythromelalgia Digital gangrence

Diagnosis Reactive ThrombocytosisRountine blood count History ( URI,IDA,Surgery,Hemorrhage,Malignancy ) Elevated IL,APP,GM-CSF ANA,RA Anemia workup Tumor marker

Complication Reactive thrombocytosisUnderlying disease Kawasaki disease: at risk of coronary thrombosis or rupture

TreatmentClonalHydroxyurea Anagrelide IFN Phosphorus-32 Low dose Aspirin BMT

ReactiveTreat underlying No need unless platelet >1,000,000 Aspirin for Kawasaki

Mean ({+/-}SD) Platelet Counts of 114 Patients with Essential Thrombocythemia Treated with Hydroxyurea or Left Untreated

Cortelazzo S et al. N Engl J Med 1995;332:1132-1137

Probability of Thrombosis-free Survival in 114 Patients with Essential Thrombocythemia Treated with Hydroxyurea or Left Untreated

Cortelazzo S et al. N Engl J Med 1995;332:1132-1137

ET - natural history Rarely progresses to AML (progression in