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Platelet
Granule :
Glycoprotein Thrombospondin VEGF PDGF
Angiogenesis
Dense granule :
Ca Serotonin ADP Thromboxane A2 Aggregation
Platelet aggregation
Definition of 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
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
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
Probability of Thrombosis-free Survival in 114 Patients with Essential Thrombocythemia Treated with Hydroxyurea or Left Untreated
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