myeloid cancers

Question 1

How can polycythemia and myeloproliferative disorders be classified?

Answer 1

Polycythemia can be relative (lack of plasma) or true (due to excess rbc)

Myeloproliferative neoplasms can be philadelphia positive or negative

Question 2

If there is polycythemia aka raised haemoglobin count or raised haematocrit, what do you need to think?

Answer 2

45-48 percent should normally be red blood cell

Is there pseudopolycythemia aka a relative deficiency of plasma

Dilution studies or Fick principle seeks to understand the ratio of albumin (plasma) to rbc ratio by radiolabelling

Question 3

What might cause pseudopolycythmia?

Answer 3

Alcohol
Obesity
Diuretics

Question 4

If we have established that there is polcythemia, how do we subclassify it?

Answer 4

Secondary - raised EPO
Primary - low EPO

Question 5

EPO can be raised appropriately or inappropriately. What are causes of appropriate high EPO?

Answer 5

High altitude (low oxygen causing chronically high EPO)
Hypoxic lung disease
Cyanotic heart disease (left to right shunt)
High affinity haemoglobin (which fails to release oxygen)

Question 6

EPO can be raised appropriately or inappropriately. What are causes of appropriately high EPO?

Answer 6

High altitude (low oxygen causing chronically high EPO)
Hypoxic lung disease
Cyanotic heart disease (left to right shunt)
High affinity haemoglobin (which fails to release oxygen)

Question 7

EPO can be raised appropriately or inappropriately. What are causes of inappropriate high EPO?

Answer 7

Renal disease - cysts, tumours e.g. medulloblastomas (renal cancers) or inflammation

Uterine myoma
Other tumorus

Question 8

What can haematopoetic stem cells

Answer 8

Common haematopoetic stem cell can become granulocyte macrophage precursor, megakarocyte precursor and also red blood cell precursors

Question 9

Polycythemia vera is associated with which mutaiton?

Answer 9

JAK2 - myelofibrosis

Others include calcitrieun??????? and mpl????????

Question 10

diagnosis of

Answer 10

Note:organomegaly is the key clnicla
Hepatosplenomegaly

FBC
BM biopsy
EPO
??

Question 11

Symptoms of PCV

Answer 11

Headaches
Light headedness
TIA
storke
Visual disturbances
Fatigue
Dysnpoea

Increased histamine released
Pepetic ulceration
Aqua pruritis (after a hot bath)

Question 12

Principles of treatment in PCV

Answer 12

reduce HCT through venesection or cytoreductive therapy - hydroxycarbamide

reduce risk of thrombosis

Question 13

Principles of treatment in PCV (myeloproliferative disorder of erythrocytes)

Answer 13

reduce HCT through venesection or cytoreductive therapy - hydroxycarbamide

reduce risk of thrombosis

Question 14

Essential thromobcythemia (myeloproliferative disorder of platelets) epidemiology

Answer 14

Peaks in 55 or 30 years of age
M=F

e.g. around 400/500 platelets for no reason - picked up on normal scan

Question 15

ET symptoms

Answer 15

Hypercoagulability
Strokes
Gangrenes
TIAs

Bleeding due to defects - mucous membrane and cutaneous bleeding

Modest enlargement of spleen

Question 16

ET treatment

Answer 16

Give aspirin to prevent thrombosis
Hydroxycarbamide to bring platelet count down

Anagrelide - specific inhibition of platelets

Question 17

What is the prognosis of ET

Answer 17

5 percent chance of acquiring new mutations (leukemic transformation)

prognosis good

Question 18

Phil negative myeloproliferative disorder - PRIMARY MYELOFIBROSIS

Answer 18

Abnormal myeloproliferation associated with fibroblast growth stimulating factor, causing reactive bone marrow fibrosis

Question 19

Phil negative myeloproliferative disorder - PRIMARY MYELOFIBROSIS

Answer 19

Abnormal myeloproliferation associated with fibroblast growth stimulating factor, causing reactive bone marrow fibrosis

m=f

Question 20

main clinical symptom of primary myelofibrosis and why

Answer 20

MASSIVE SPLENOMEGALY
- budd chiari syndrome

as the BM starts to have collage deposition, so haemopoesis starts to occur in other organs where it can happen - e.g. liver and spleen

e.g. as fetuses we made blood cells in the liver as kids

Dry bone tap

Question 21

Primary myelofibrosis treatments

Answer 21

Not great

Supportive treatment - RBCs, platelet transfusion ineffctive due to splenomegaly

Question 22

Philadelphia POSITIVE myeloproliferative disorders

Answer 22

CML
- radiation is a major risk factor

Question 23

CML presentation

Answer 23

Tiredness
Hypermetabolism
Thrombotic events
Monocular blindness
Bleeding upon bruising

Question 24

Examination of CML

Answer 24

Massive splenomegaly
Hepatomegaly

Question 25

What does JAk2 indicate?

Answer 25

JAK 2 positive = polycythemia vera

Question 26

Blastic transformation is also known as?

Answer 26

Imitanib helps, but then a year later bloods have crashed, and