Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology Revision > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What are your myeloproliferative diseases associated with?

A

Myeloid Leukaemia!!

They are a precursor

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

What are these caused by?

A

Proliferation os a cline of haematopoietic myeloid stem cells in the marrow, they retain the ability to differentiate in the following cells:

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

What are the 4 types of cells that they can effect?

A

RBC
WBC
Platelets and Fibrobalsts

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

What is the myleoproliferation of RBC causing?

A

Polycythaemia Rubra Vera

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

Prolif of WBC’s?

A

CML

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

Platelets led to?

A

Essential thrombocytopenia

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

What do fibroblast proliferation lead to?

A

Myelofibrosis

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

What is Polycythaemia Rubra Vera?

A

This is a malignant proliferation of a clone derived form one of the pluripotent stem cells

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

What mutation is PRV asoacited with?

A

JAK2 - V61F in 95%

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

Whta re the 3 big pathophysiological process os PRV?

A

Proliferation of RBC leading to hyper viscosity and then thrombosis

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

What are the symptoms of PRV

A

Headaches, itch, burin in the digits, facial plethora, splenomegaly, thrombosis, TIA, stoke, AT and VT, ischaemia, gout due ti increased uric acid

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

What are the key investigating of PRV?

A

Blood film and count

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

What will the blood show in PRV?

A

Increased RBC’s, heamtocrit and Hb with higher than normal WCC and platelets with a high uric acid and a try increase in the volume of RBC’s

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

what are the principles of management of PRV?

A

Venesecion in the fit

apron dn hydroxycarbamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the complications of PRV?

A

Stroke, TIA, bone marrow failure and AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What percentage go onto AML?

A

5%

17
Q

What is the aim of Management?

A

To keep the haemocrit to 0.45M

18
Q

What is the definition of essential thrombocytopenia?

A

This is a myeloproliferative disorder where the is clinical proliferation fo the megakaryocytic causing production of high and abnormal platelets, leading to coagulaopathy

19
Q

What mutation is associated with ET?

A

JAK2 V61F

20
Q

What is the predominant cell that proliferates in ET?

A

Platelets

21
Q

What are the clinical presentations of ET?

A

Arterial and venous thrombosis
Digital ischaemia(erythromegalia)
Gout
Mild splenomegaly

22
Q

What is seen on blood count in ET?

A

Very high platelets >1000x 10^9

23
Q

What is the medical management of choice of ET?

A

Low dose aspirin and if pregnant then hydroxycarbamide

24
Q

What are the 2 complications of ET?

A

Myelofibrosis and AML

25
Q

What is myelofibrosis?

A

This is hyperplasia of the megakaryocytes causing production of PDFG - platelet derived growth factor which causes marrow fibrosis and myeloid dysplasia

26
Q

What is THE characteristic sign of myelofibrosis?

A

Massive HEPATOSPLENOMEGALY due to haemopoiesis in the spleen and liver

27
Q

What are the clinical features? of MF?

A

Hypermetabolic syndromes = weight loss, fever, night sweats
Abdominal discomfort
Bone marrow failure

28
Q

what is the key diagnostic test and what will it show in MF?

A

FBC and blood film” Tear drop RBCs and erythroblasts
low Hb
Bone marrow trephine for Dx

29
Q

What are the principles of management of MF?

A

Marrow support ie RBC and platelet transactions

allogenic stem cell transplant

30
Q

What are the complications fo MF?

A

AML

31
Q

What is myelodysplasia?

A

Aquired clonal disorder of the bone marrow

32
Q

What is the main risk macros of MD?

A

Old age

33
Q

What is MD a precursor of?

A

AML and bone marrow failure

34
Q

What does the FBC and blood film show in MD?

A

Macrocytic anaemia and pancytopenia with or without B12/folate deficiency

35
Q

How are you gong to manage people with MD?

A

Bone marrow support ie RBs and platelet transfusions
Erythropoietin and G-CSF
Immunosuppression ie Ciclosporin
allogenic stem cell transplants

36
Q

What are the complications os MD?

A

Bone marrow failure and AML

Iron accumulation from many transfusions

37
Q

What percentage of people with MD get AML?

A

33.33333%

Haematology Revision (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions