Myeloproliferative Disorders

Question 1

What are your myeloproliferative diseases associated with?

Answer 1

Myeloid Leukaemia!!

They are a precursor

Question 2

What are these caused by?

Answer 2

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

Question 3

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

Answer 3

RBC
WBC
Platelets and Fibrobalsts

Question 4

What is the myleoproliferation of RBC causing?

Answer 4

Polycythaemia Rubra Vera

Question 5

Prolif of WBC’s?

Answer 5

CML

Question 6

Platelets led to?

Answer 6

Essential thrombocytopenia

Question 7

What do fibroblast proliferation lead to?

Answer 7

Myelofibrosis

Question 8

What is Polycythaemia Rubra Vera?

Answer 8

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

Question 9

What mutation is PRV asoacited with?

Answer 9

JAK2 - V61F in 95%

Question 10

Whta re the 3 big pathophysiological process os PRV?

Answer 10

Proliferation of RBC leading to hyper viscosity and then thrombosis

Question 11

What are the symptoms of PRV

Answer 11

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

Question 12

What are the key investigating of PRV?

Answer 12

Blood film and count

Question 13

What will the blood show in PRV?

Answer 13

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

Question 14

what are the principles of management of PRV?

Answer 14

Venesecion in the fit

apron dn hydroxycarbamide

Question 15

What are the complications of PRV?

Answer 15

Stroke, TIA, bone marrow failure and AML

Question 16

What percentage go onto AML?

Answer 16

5%

Question 17

What is the aim of Management?

Answer 17

To keep the haemocrit to 0.45M

Question 18

What is the definition of essential thrombocytopenia?

Answer 18

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

Question 19

What mutation is associated with ET?

Answer 19

JAK2 V61F

Question 20

What is the predominant cell that proliferates in ET?

Answer 20

Platelets

Question 21

What are the clinical presentations of ET?

Answer 21

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

Question 22

What is seen on blood count in ET?

Answer 22

Very high platelets >1000x 10^9

Question 23

What is the medical management of choice of ET?

Answer 23

Low dose aspirin and if pregnant then hydroxycarbamide

Question 24

What are the 2 complications of ET?

Answer 24

Myelofibrosis and AML

Question 25

What is myelofibrosis?

Answer 25

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

Question 26

What is THE characteristic sign of myelofibrosis?

Answer 26

Massive HEPATOSPLENOMEGALY due to haemopoiesis in the spleen and liver

Question 27

What are the clinical features? of MF?

Answer 27

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

Question 28

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

Answer 28

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

Question 29

What are the principles of management of MF?

Answer 29

Marrow support ie RBC and platelet transactions | allogenic stem cell transplant

Question 30

What are the complications fo MF?

Answer 30

AML

Question 31

What is myelodysplasia?

Answer 31

Aquired clonal disorder of the bone marrow

Question 32

What is the main risk macros of MD?

Answer 32

Old age

Question 33

What is MD a precursor of?

Answer 33

AML and bone marrow failure

Question 34

What does the FBC and blood film show in MD?

Answer 34

Macrocytic anaemia and pancytopenia with or without B12/folate deficiency

Question 35

How are you gong to manage people with MD?

Answer 35

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

Question 36

What are the complications os MD?

Answer 36

Bone marrow failure and AML | Iron accumulation from many transfusions

Question 37

What percentage of people with MD get AML?

Answer 37

33.33333%