Myeloproliferative Disorder: Essential Thrombocythemia Flashcards
What is ET?
A clonal proliferation of megakaryocytes - leads to persistently raised platelets
The platelets have abnormal function. True or false?
True
In ET what range are the platelets usually above?
Often more than 1000x10*9 /L
What is there a risk of?
Haemorrhage
Arterial or venous thrombosis
Microvascular occlusion - headache, atypical chest pain, light headedness
What should be excluded?
Other causes of thrombocytosis
What other causes of thrombocytosis are there?
May be a reaction phenomenon seen with many conditions, including: Bleeding Infection Chronic inflammation Malignancy Trauma Post surgery Iron deficiency
How is it treated?
Aspirin 75mg OD
Hydroxycarbamide in high risk patients (chemotherapy)
What is there a risk of transformation to?
Myelofibrosis and AML
What is the average age that it affects?
60 years
What symptoms are associated?
May be asymptomatic Burning on soles or palms Cold peripheries Headache Dizziness
Haemorrhagic complications: epistaxis, ecchymosis, menorrhagia
Splenomegaly
50% have acquired what mutation?
JAK2