Haematology 4: Myeloproliferative Disorders Flashcards
What are the main differences between myeloproliferative and myelodysplastic disorders ?
Myelodysplastic syndromes have ineffective differentiation whereas in myeloproliferative disorders the cells are normal and fully differentiated.
Give 4 examples of myeloproliferative disorders ?
Polycythaemia rubra Vera
Essential thropmbocythaemia
Idiopathic myelofibrosis
Idiopathic erythrocytosis
List 4 signs/symptoms of Polycythaemia rubra Vera ?
Aquagenic pruritus- itch after having a warm bath
Gout
Engorged retinal veins
Erythromelalgia- Red painful extremities
What levels would you expect in someone with Polycythaemia rubra Vera ?:
Hb :Low/High
Hct :Low/High
MCV: Low/High
EPO: Low/High
Hb: High
Hct: High
MCV: High
EPO: low (suppressed because of negative feedback)
How is Polycythaemia rubra Vera diagnosed ?
Bone marrow biopsy- Increased cellularity, low EPO, Reticulin fibrosis
JAK2 V617F mutation is diagnostic
What is pseudo polycythaemia ?
Reduced plasma volume in presence of a normal Hb conc.
What causes Polycythaemia where EPO is raised ?
Hypoxia- High altitude
Renal disease
tumours
What are the signs of Essential Thrombocythaemia ?
High platelet count
CVA
DVT/PE
Splenomegaly
What are the signs of Chronic idiopathic myelofibrosis ?
FLAWS
anaemia
Thrombocytosis
Hepatosplenomegaly (massive, sites for extra medullary haematopoeisis)
What is the characteristic finding of bone marrow aspiration in Chronic idiopathic myelofibrosis
Dry tap
Because of the fibrosis nothing comes out