Brainscape's Knowledge GenomeTM

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


See full index

Path - Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What cell do myeloproliferative disorders arise from?

A

Common myeloid progenitor cell

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

How is haematopoesis controlled?

A

Through interaction between GF and GF receptors

This interaction activates TKs

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

Which kinases play an important role in Ph- MPD?

A

JANUS KINASES (JAK)

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

How do GF control haematopoesis?

A

GF binds to GF receptor
This activates JAK
JAK causes STAT pathway activation
This leads to cell growth and differentiation

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

What occurs in myeloproliferation in terms of differentiation and proliferation?

A

Differentiation and Proliferation occur

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

What occurs in leukaemia in terms of differentiation and proliferation?

A

Proliferation

Little/no differentiation

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

What occurs in myelodysplastic syndrome in terms of differentiation and proliferation?

A

INEFFECTIVE proliferation and differentiation

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

Which mutation triggers polycythaemia vera?

A

JAK2 V617F

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

What occurs in polycythaemia vera?

A

Increased production of RBC (high Hb, Htc)
Independent of mechanisms that regulate EPO
There is also a compensatory increase in plasma volume

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

What are symptoms of polycythaemia vera?

A

Hyperviscosity:

  • headache, dizziness, stroke
  • visual disturbance
  • fatigue, dyspnoea

Increased histamine release

  • aquagenic pruritus
  • peptic ulceration
Splenomegaly 
Plethora
Eruthromelalgia 
Thrombosis 
Retinal vein engorgement 
gout (due to increased RBC turnover, causing increased uric acid production)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How do you investigate polycythaemia vera?

A

FBC
Bone marrow biopsy(increased cellularity)
Low EPO (suppression via neg feedback loop)
JAK2 V617F mutation (DIAGNOSTIC)
Isotope dilution

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

What should you consider if a patient with polycythaemia clinical picture is JAK2 negative?

A

Consider pseudopolycythaemia

Consider secondary polycythaemia

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

What is treatment for polycythaemia vera?

A

Reduce viscosity (Hct <45%)

  • Venesection
  • Cytoreductive therapy

Reduce thrombotic risk

  • Aspirin
  • Platelets <400
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is idiopathic erythrocytosis?

A

Isolated erythrocytosis (with low EPO)
NO JAK2 V617F mutation
Occasionally JAK2 Exon 12 mutation

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

Whats the prognosis of idiopathic erythrocytosis?

A

Normal life

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

What are causes of death in PV?

A

Thrombosis
Leukaemia
Myelofibrosis

17
Q

What is essential thrombocythaemia?

A

Sustained thrombocytosis >600

Involves PLATELETS ONLY

18
Q

What is the clinical presentation of essential thrombocythaemia?

A

INCIDENTAL FINDING
Thrombosis (CVA, gangrene, TIA, DVT/PE)
Bleeding
Headache,dizziness, visual disturbance

19
Q

What are diagnostic criteria for essential thrombocythaemia?

A

Platelet count above 600
Mekacaryocyte clustering
No evidence of reactive thrombocytopenia ptosis (infection)
JAK2 V617 mutation

20
Q

In what ratio of essential thrombocythaemia is JAK2 V617F mutation present?

A

50% of patients

21
Q

What is the treatment for ET?

A

Aspirin (prevents thrombosis)
Anagrelide
Hydroxycarbamide
Alpha-interferon

22
Q

What is chronic myelofibrosis?

A

Chronic myeloproliferative disease with proliferation of megakaryocytic and granulocytic cells
Causes reactive bone marrow fibrosis and extramedullary haematopoesis

23
Q

Clinical presentation of myelofibrosis

A

anaemia, thrombocytopenia
splenomeg/hepatomeg
Hypermetabolic state

24
Q

Stages of myelofibrosis

A

Pre-fibrotic: hypercellular marrow, mild blood changes

Fibrotic: DRY TAP, splenomegaly, collagen fibrosis, osteosclerosis

25
Q

What are blood film findings for myelofibrosis?

A

Leukoerythroblastic picture (nucleated red cells)
Tear drop poikilocytes
Giant platelets
Circulating megakaryocytic

26
Q

How is the bone marrow investigated in myelofibrosis=

A

Dry tap

Trephine biopsy

27
Q

How is myelofibrosis treated? Based on presentation

A

Anaemia > transfusion
Platelet transfusion
Splenecomy
Cryoreductive therapy: hydroxycarbamide, thalidomide

Path - Haematology (17 decks)

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