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Thrombocytosis

dr Putra Hendra SpPD


UNIBA

Platelet
Granule :
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 Thrombocytosis
Rare 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 ?
Clonal
myeloproliferative dysregulation malignant changes thrombotic/hemo-rrhagic complication

Reactive
secondary responses physiological feedback transient and subsides exceedingly rare/ Kawasaki disease

Clonal
mpl

Reactive
P P
TPO IL-6 TPO IL-3 IL-11 GM-CSF EPO

Sensitivity to TPO

Mega

Mega

Proliferation

Proliferation

DiagnosisClonal thrombocythemia
Blood Smear
Myelocyte Metamyelocyte Thrombocyte

Bone Marrow aspirate/biopsy


Giant megakaryocyte Hypercellularity Hyperplasia

Diagnosis Clonal thrombocythemia


Cytogenetic study
CML,Ph1,bcr-abl

Platelet aggregation test


ADP,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 thrombocytemia
Neurologic:
Headache TIA

Thrombosis:
Coronary Renal Portal DVT, Pul

Bleeding:
GI Skin Eye Brain Urinary tract

ComplicationClonal thrombocytemia
Pregnancy:
Spontaneous abortion IUGR

Others:
Erythromelalgia
Digital gangrence

Diagnosis Reactive Thrombocytosis


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

Complication Reactive thrombocytosis


Underlying disease Kawasaki disease: at risk of coronary thrombosis or rupture

Treatment
Clonal
Hydroxyurea Anagrelide IFN Phosphorus-32 Low dose Aspirin BMT

Reactive
Treat 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 <1% of pts) May progress to myelofibrosis Major complication is thrombosis
Occurs in 20-30% of patients Clots may be arterial or venous