Haematology 4: Myeloproliferative Disorders Flashcards

1
Q

What are the main differences between myeloproliferative and myelodysplastic disorders ?

A

Myelodysplastic syndromes have ineffective differentiation whereas in myeloproliferative disorders the cells are normal and fully differentiated.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Give 4 examples of myeloproliferative disorders ?

A

Polycythaemia rubra Vera
Essential thropmbocythaemia
Idiopathic myelofibrosis
Idiopathic erythrocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

List 4 signs/symptoms of Polycythaemia rubra Vera ?

A

Aquagenic pruritus- itch after having a warm bath
Gout
Engorged retinal veins
Erythromelalgia- Red painful extremities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What levels would you expect in someone with Polycythaemia rubra Vera ?:

Hb :Low/High
Hct :Low/High
MCV: Low/High
EPO: Low/High

A

Hb: High
Hct: High
MCV: High
EPO: low (suppressed because of negative feedback)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is Polycythaemia rubra Vera diagnosed ?

A

Bone marrow biopsy- Increased cellularity, low EPO, Reticulin fibrosis

JAK2 V617F mutation is diagnostic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is pseudo polycythaemia ?

A

Reduced plasma volume in presence of a normal Hb conc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What causes Polycythaemia where EPO is raised ?

A

Hypoxia- High altitude
Renal disease
tumours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the signs of Essential Thrombocythaemia ?

A

High platelet count
CVA
DVT/PE
Splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the signs of Chronic idiopathic myelofibrosis ?

A

FLAWS
anaemia
Thrombocytosis
Hepatosplenomegaly (massive, sites for extra medullary haematopoeisis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the characteristic finding of bone marrow aspiration in Chronic idiopathic myelofibrosis

A

Dry tap

Because of the fibrosis nothing comes out

How well did you know this?
1
Not at all
2
3
4
5
Perfectly