Brainscape's Knowledge GenomeTM

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


See full index

Haematology Week 3 2018/19 > 8: Myeloproliferative disorders > Flashcards

8: Myeloproliferative disorders Flashcards

1
Q

What is the myeloid lineage?

A

Granulocyte

Red blood cell

Platelet

lineages

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

What is the difference between chronic myeloid leukaemia and acute myeloid leukaemia?

A

Differentiation and maturation PRESERVED in chronic disease

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

Which mutation is associated with chronic myeloid leukaemia?

A

Philadelphia chromosome

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

CML is BCR-ABL1 (positive / negative).

A

CML = BCR-ABL1 positive

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

What is a normal, secondary cause for a rise in blood components?

A

Reactive change

in response to infection usually

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

Which blood components can increase in myeloproliferative disorders?

A

RBCs

Platelets

White cells (basophils/eosinophils is especially significant)

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

Which organ can enlarge in myeloproliferative disorders?

A

Spleen

Increased cell breakdown

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

What is a risk seen in hyperviscosity secondary to raised cell counts?

A

Thrombosis

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

Chronic ___ causes a secondary polycythaemia - why?

A

Chronic hypoxia (e.g sleep apnoea, COPD)

Persistent EPO release by the kidneys

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

What is the course of untreated chronic myeloid leukaemia?

A

1. Chronic phase for 3-5 years, maturation intact

2. Accelerated phase - maturation starting to fail

3. Blast crisis and death - more closely resembling an acute leukaemia

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

What are some clinical features of CML?

A

Hyperviscosity symptoms

Hypermetabolism - cachexia

Splenomegaly

Gout

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

How is suspected CML investigated?

A

FBC

Bone marrow biopsy

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

An increase in which specific blood components strongly suggest CML?

A

Eosinophilia

Basophilia

not normally found in large numbers

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

What might be misdiagnosed as CML based on blood changes?

A

Reactive changes due to infection

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

Which genetic mutation is commonly found in CML?

A

Unbalanced chromosome 22 translocation

‘BCR-ABL’ gene on the Philadelphia chromosome

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

BCR-ABL mutations on the Philadelphia chromosome affect which protein?

A

Tyrosine kinase

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

What is a tyrosine kinase inhibitor used to treat CML?

A

Imatinib

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Imatinib is a ___ ___ inhibitor which massively increases life expectancy in which disease?

A

tyrosine kinase inhibitor

CML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

CML is positive for which mutation?

A

BCR-ABL1

Philadelphia chromosome (22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is erythromelalgia?

Which group of diseases is it a feature of?

A

Hot, swollen, red, painful digits due to blood stasis

Myeloproliferative diseases

Caused by HYPERVISCOSITY of blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

In which disease does haemoglobin and haematocrit increase abnormally?

A

Polycythaemia

22
Q

What is primary polycythaemia called?

A

Polycythaemia rubra vera

23
Q

Which genetic mutation is implicated in polycythaemia rubra vera?

A

JAK2

24
Q

What causes secondary polycythaemia?

A

Chronic hypoxia

EPO-secreting renal tumours

25
Q

What is pseudopolycythaemia?

A

Appearance of raised Hb / haematocrit which is actually due to a reduced plasma volume

26
Q

What causes pseudopolycythaemia?

A

Dehydration / Diuretic therapy

Obesity

Alcohol excess

27
Q

Pseudopolycythaemia and ___ polycythaemia mean the same thing.

A

apparent polycythaemia

28
Q

What are some symptoms of primary polycythaemia?

A

Plethora

Itch (caused by warm water)

Hyperviscosity symptoms

29
Q

Which genetic mutation is screened for in polycythaemia rubra vera?

A

JAK2

30
Q

JAK2 mutations are seen in 95% of patients with ___ ___ ___.

A

polycythaemia rubra vera (primary)

31
Q

What process is uninhibited it patients with polycythaemia rubra vera?

A

Erythropoiesis

32
Q

What are some investigations for suspected secondary polycythaemia?

A

CXR

Medication history - e.g testosterone

EPO levels

Bone marrow biopsy

33
Q

How is polycythaemia rubra vera treated?

A

Venesection until haematocrit is < 0.45

Aspirin (anti-platelet - deactivates platelets, reducing hyperviscosity of blood)

Cytotoxic drugs e.g hydroxycarbamide - kills off excess cells

34
Q

Polycythaemia rubra vera can lead to thrombosis in some patients.

How is this prevented?

A

Aspirin

35
Q

In addition to venesection, how can Hb / haematocrit be reduced in polycythaemia rubra vera?

A

Cytotoxic drugs e.g hydroxycarbamide

36
Q

In which disease is idiopathic platelet excess seen?

A

Essential thrombocythaemia

37
Q

Essential thrombocythaemia can cause both thrombosis and abnormal bleeding.

Why?

A

Thrombosis - excess of platelets

Abnormal bleeding - platelets mop up vWF, causing acquired von Willebrand’s disease

38
Q

Is anaemia a feature of essential thromocythaemia?

A

No

Don’t confuse ET for thrombocytosis caused by reactive change, in which patient would be anaemia

39
Q

RBCs and platelets are derived from the same progenitor cells.

What are some causes of reactive thrombocytosis which may cause a blood picture similar to essential thrombocythaemia?

A

Blood loss

Haemolysis

Inflammation

Malignancy

40
Q

How is essential thrombocythaemia treated?

A

Aspirin (anti-platelet drug)

Chemotherapy to suppress proliferation in some patients

41
Q

Which myeloproliferative disorder causes progressive fibrosis of the bone marrow?

A

Myelofibrosis

42
Q

Myelofibrosis can occur secondary to which other MPDs?

A

Polycythaemia

Essential thrombocythaemia

43
Q

What blood film appearance, caused by leakage of progenitor cells into the blood, is seen in myelofibrosis?

A

Leukoerythroblastic blood film

44
Q

Which shape of RBC is seen in myelofibrosis?

A

Teardrop cells

45
Q

What are the main clinical features of myelofibrosis?

A

Pancytopaenia - anaemia, abnormal bleeding, increased infection risk

Splenomegaly

Hypercatabolism - cachexia

Teardrop cells on a blood film

46
Q

What are the two blood film appearances to look out for in myelofibrosis?

A

Leukoerythroblastic film

Teardrop cells

47
Q

What are some causes of a leukoerythroblastic blood film?

A

Reactive change as in infection

Bone marrow infiltration

Myelofibrosis

48
Q

What is the appearance of myelofibrosis on a bone marrow biopsy?

A

Fibrosis - collagen fibres

49
Q

How is myelofibrosis treated?

A

Supportive management of pancytopaenia (blood transfusion, platelet transfusion, prophylactic antibiotics)

Young, fit patients receive stem cell transplants

50
Q

Most raised blood counts will be caused by ___ change.

A

reactive change

infection

51
Q

Which supplement, often taken by bodybuilders, can cause secondary polycythaemia?

A

Testosterone

52
Q

Patients with polycythaemia rubra vera have blood taken until their haematocrit is below which threshold?

A

< 0.45

0.45 means that 45% of your blood sample is made up of red cells

Haematology Week 3 2018/19 (10 decks)

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