Thrombocytosis uniba 16-1-13
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Transcript of Thrombocytosis uniba 16-1-13
Thrombocytosis
dr Putra Hendra SpPD
UNIBA
PlateletαGranule :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
TPO
Sensitivity to TPO
Reactive
TPO
IL-6
IL-3
IL-11
GM-CSF
EPO
P P
MegaMega
Proliferation Proliferation
Diagnosis…Clonal 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
Complication…Clonal thrombocytemia…Neurologic: …Headache …TIA
…Thrombosis: …Coronary …Renal …Portal …DVT, Pul
…Bleeding: …GI …Skin …Eye …Brain …Urinary tract
…Pregnancy: …Spontaneous abortion
…IUGR
…Others: …Erythromelalgia
… Digital gangrence
Complication…Clonal thrombocytemia
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
TreatmentClonal…Hydroxyurea…Anagrelide…IFNα…Phosphorus-32…Low dose Aspirin…
…BMT
Reactive…Treat underlying…No need unless
platelet >1,000,000…Aspirin for Kawasaki
Cortelazzo S et al. N Engl J Med 1995;332:1132-1137
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
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