Brainscape's Knowledge GenomeTM

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


See full index

Haematology > Myeloproliferative Neoplasm > Flashcards

Myeloproliferative Neoplasm Flashcards

1
Q

Define myeloproliferative neoplasm

A

Clonal haemopoietic stem cell disorders which an increased production of one or more types of haemopoietic cells

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

How is MDN different to leukaemia?

A

Results in overproduction of mature differentiated cells

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

Describe the appearance of acute leukaemia microscopically

A

Monomorphic appearance with large cells and big nuclei - primitive cells

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

Describe the appearance of MPN microscopically

A

Lots of nucleated cells - hypercellular, variety of cells at different stages of maturation

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

Name the two subtypes of MPN

A

BCR ABL 1 positive

BCR ABL 1 negative

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

What disease is BCR ABL 1 positive?

A

Chronic myeloid leukaemia - overproduction of granulocytes

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

What diseases are BCR ABL 1 negative?

A

Polycythaemia vera
Essential thrombocytopenia
Primary myelofibrosis

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

What blood results would suggest MPN?

A 
High granulocyte count 
High red cell count/Hb
High platelets 
Eosinophilia/Basophilia 
Splenomegaly 
Thrombosis in unusual place
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is chronic myeloid leukaemia?

A

Proliferation of myeloid cells

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

Name the phases of chronic myeloid leukaemia

A

Chronic
Accelerated
Blast

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

Describe the chronic phase of CML

A

Predominantly mature cells - intact maturation followed by a blast crisis similar to acute leukaemia with maturation defect

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

Describe the accelerated phase of CML

A

Transition phase, high proportion of less mature cells

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

State the clinical features of CML

A 
Asymptomatic 
Splenomegaly (LUQ discomfort, early satiety) 
Hypermetabolic syndrome 
Gout 
Priapism - hyperleukocytosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Name the investigations done in CML

A

Blood count and film
Bone marrow sample
Philadelphia chromosome

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

Describe the blood film in CML

A

Normal/reduced Hb, leukocytosis with neutrophilia

Myeloid precursors, eosinophilia, basophilia, thrombocytosis

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

What will a bone marrow sample in CML look like?

A

Increased cellularity

Increased granulocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the philadelphia chromosome?

A

Translocation chromosome

Results in fusion of ABL gene and BCR gene on chromosome 11 - tyrosine kinase which causes abnormal phosphorylation

18
Q

How is CML treated?

A

Tyrosine Kinase Inhibitor

19
Q

What are the common features of BCR ABL 1 negative MPNs?

A 
Asymptomatic 
Increased cellular turnover - gout, fatigue, weight loss, sweats
Splenomegaly 
Marrow failure 
Thrombosis 
Erythromelagia
20
Q

What is erythromelagia?

A

Pain in hands and feet due to microvascular occlusion

21
Q

What is polycythaemia vera?

A

MPN characterised by high Hb and haematocrit accompanied by erythrocytosis

22
Q

Describe the difference between a true and pseudo polycythaemia

A

True - increase in proportion of red cells

Pseudo - normal red cells but reduced plasma volume causes increased Hct

23
Q

What causes secondary polycythaemia?

A

Hypoxia
Smoking
Tumours - erythropoietin secreting

24
Q

What causes pseudopolycythaemia?

A

Dehydration
Obesity
Diuretics

25
Q

State the features of polycythaemia vera

A

Headache
Fatigue
Itch - aquagenic puritis

26
Q

How is polycythaemia vera investigated?

A

FBC, blood film, look for secondary cause

JAK2 mutation - results in loss of auto-inhibition activation of erythropoiesis in the absence of ligand

27
Q

How is JAK2 mutation identified?

A

Genetic analysis of peripheral blood

28
Q

Describe the treatment of polycythaemia vera

A

Prevent thrombosis
- venesect to haematocrit
- aspirin
Cytotoxic chemotherapy

29
Q

What is essential thrombocythaemia?

A

Over production of abnormal platelets

30
Q

Why can essential thrombocythaemia cause bleeding?

A

Acquired Von Willebrand disease - vWF absorbed onto the surface of abnormal platelets and reduces expression

31
Q

Name the clinical features of essential thrombocythaemia

A

Vaso-occlusive complications

Bleeding

32
Q

What diseases can present like essential thrombocythaemia?

A

CML

Reactive thrombocytosis

33
Q

How do you investigate essential thrombocythaemia?

A

Genetics - JAK2, CALR, MPL

Bone marrow - increased and abnormal megakaryocytes

34
Q

How is essential thrombocythaemia treated?

A

Anti-platelet aspirin
Cytoreductive therapy to control proliferation -
- hydroxycarbamide, IFN alpha

35
Q

State the characteristics of myelofibrosis

A 
Marrow failure 
Bone marrow fibrosis 
Extramedullary hematopoiesis 
Leukoerythroblastic film 
Teardrop RBC
36
Q

What are the clinical features of myelofibrosis?

A

Marrow failure
Splenomegaly
Hypercatabolism

37
Q

How is myelofibrosis diagnosed?

A

Blood film
Dry aspirate/trephine biopsy
Genetics

38
Q

What classical RBC shape is seen on blood film?

A

Poikilocytes teardrop RBC in peripheral blood

39
Q

What is leukcoerythroblastic film?

A

Presence of neutrophil and red cell precursors in the peripheral blood

40
Q

What causes leukcoerythroblastic film?

A

Reactive - sepsis
Marrow infiltration
Myelofibrosis

41
Q

What will a trephine biopsy of myelofibrosis show?

A

Loss of fat spaces and dense network of reticulin fibres

42
Q

How is myelofibrosis treated?

A

Supportive - transfusion, antibiotics
Allogenic stem cell transplant - potentially curable
Splenectomy
JAK 2 inhibitor

Haematology (18 decks)

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