myeloproliferative disorders 250821 Flashcards

1
Q

true vs relative polycythaemia

A
relative = plasma volume is low 
true = red cells increased plasma normal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

true polycythaemia causes in JAK2 mutation negative

A

increased EPO due to hypoxia or renal disease tumours, familial

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

raised haematocrit JAK positive

A

polycythaemia or erythrocytosis

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

JAK2 V617F

A

polycythaemia vera

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

polycythaemia vera definition and symptoms

A

independent not EPO driven increased production of RBCs accompanied by different degrees of increase in platelets or granulocytic cells
symptoms = headaches, light headedness, stroke, visual disturbances, fatigue, dyspnoea, increased histamine release - aquagenic pruritus, peptic ulceration, splenomegaly, plethora, eryhtomelalgia, gout thrombosis

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

investigations polycythaemia

A

high Hb, high plasma volume , high Hct, No circulating immature cells, high MCV, white cells normal

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

essential thrombocythaemia

A

mainly involves megakaryocyte lineage, sustained thrombocytosis, thrombosis, bleeding, headaches, dizziness, visual disturbances,

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

diagnostic criteria for essential thrombocythaemia

A

platelet count consistnetly above 600x 10^6, megakaryocyte abnormalities,

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

chronic idiopathic myelofibrosis

A

clonal myeloproliferative disease with proliferation mainly of megakaryocytes and granulocytic cells associated with reactive bone marrow fibrosis and extramedullary haemopoeises

clinical presentation: thrombocytosis, cytopaenias, splenomegaly, hypermetabolic state (weight loss fatigue etc),

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

diagnosis of chronic idiopathic myelofibrosis

A
pre-fibrotic = blood changes are mild, hypercellular marrow
fibrotic = splenomegaly, blood changes, dry tap, prominent collagen fibrosis, later osteosclerosis

blood film - leucoerythroblastic, extramedullary haemopoiesis in liver and spleen, bone marrow dry tap, trephine biopsy shows increased reticulin or collagen fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Chronic myeloid leukaemia mutation

A

BCR-ABL Ph Chr mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

CML symptoms

A

lethargy, hypermatabolism, thrombotic event e.g. mono-ocular blindness, CVA, bruising, bleeding

massive splenomegaly, hepatomegaly,

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

CML bloods

A

Hb and platelets are normal or raised, massive leucocytosis , neutrophils myelocytes, basophilia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

phases of CML

A

chronic phase
accelerated phase
blast crisis blasts over 20%

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