Chronic Myeloproliferative Disorders Flashcards
What is the pathophysiology behind myeloproliferative disorders?
Abnormal proliferation of myeloid cells in the bone marrow
Which chronic myeloproliferative disorder is BCR-ABL1 positive?
Chronic myeloid leukaemia
What are some signs and symptoms which are common to all myeloproliferative disorders?
Symptoms of increased cell turnover, splenomegaly, arterial/venous thrombosis
What are some symptoms of increased cell turnover which may be seen in patients with myeloproliferative disorders?
Fatigue, weight loss, night sweats, gout
An increase in the number of which cells is highly suggestive of a myeloproliferative disorder rather than a reactive cause?
Eosinophils and basophils
When does the incidence of polycythaemia vera peak?
6th decade of life
What is the pathophysiology behind polycythaemia vera?
A high haemoglobin/haematocrit accompanied by erythrocytosis
What are the two main secondary causes of polycythaemia?
Chronic hypoxia or inappropriately high Epo production
What are some potential causes of pseudo-polycythaemia?
Dehydration, diuretic use, obesity
What are some symptoms that may be experienced by individuals with polycythaemia vera due to hyperviscosity of blood?
Headache and visual disturbances
An itch which is worsened on exposure to hot water is most suggestive of what diagnosis?
Polycythaemia vera
95% of patients with polycythaemia vera have a mutation in what protein?
JAK2
What is the first line investigation for polycythaemia vera?
JAK2 mutation status
When treating polycythaemia vera, the aim is for a haematocrit level of less than what?
0.45
What treatment is used to decrease the haematocrit in patients with polycythaemia vera who are young and low-risk?
Venesection
What treatment is used to decrease the haematocrit in patients with polycythaemia who are high-risk, i.e. aged > 60 or previous thrombotic event?
Hydroxyurea
When treating myeloproliferative disorders, what drug is preferred over the use of hydroxyurea in young women of child bearing age?
Alpha interferon
What treatment is given to patients with polycythaemia vera and essential thrombocytosis to reduce the risk of thrombosis?
Low dose aspirin
How often should FBC be monitored in patients with polycythaemia vera?
Every 3 months
The risk of both thrombosis and haemorrhage is increased in patients with which myeloproliferative disorder?
Essential thrombocytosis
A burning sensation in the hands is a characteristic feature of which myeloproliferative disorder?
Essential thrombocytosis
What mutation may be present in patients with essential thrombocytosis who are negative for JAK2?
CALR
What is the first line treatment for essential thrombocytosis?
Hydroxyurea
Myelofibrosis can be idiopathic or can develop as a result of what other conditions?
Polycythaemia vera or essential thrombocytosis
A blood film showing tear-drop shaped red blood cells (poikilocytes) is suggestive of what diagnosis?
Myelofibrosis
How is myelofibrosis diagnosed?
Bone marrow biopsy
What treatment may be curative in young patients with myelofibrosis but comes with a high risk of mortality?
Allogenic stem cell transplant
Ruxolitib is an example of what type of drug?
JAK2 inhibitor
What is the mean survival after diagnosis of myelofibrosis?
4-5 years
