Brainscape's Knowledge GenomeTM

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


See full index

Haematology (E) > Polycythaemia & Myeloproliferative disorders > Flashcards

Polycythaemia & Myeloproliferative disorders Flashcards

1
Q

What is polycythaemia?

A

Excess of red cells

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

What are myeloproliferative disorders?

A

Malignant excess of red cells

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

What may happen in a psuedo polycythaemia?

A

Decreased plasma volume

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

What patients are likely to get relative/pseudo polycythaemia?

A

Alcoholic
Obese
Diuretics

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

In what situations is raised erythropoietin appropriate?

A

High altitude
Hypoxic lung disease
Cyanotic heart disease
High affinity haemoglobin

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

What are some conditions where you get inappropriate raised erythropoietin?

A 
Renal disease (cysts, tumours, inflammation)
Uterine myoma
Other tumours (liver, lung)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the types of myeloid haematological malignancies?

A

Acute myeloid leukaemia (blasts >20%)
Myelodysplasia (blasts 5-19%)
Myeloproliferative disorders
Chronic myeloid leukaemia

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

List some myeloproliferative disorders

A 
Essential thrombocytaemia (megakaryocyte)
Polycythaemia vera (erythroid)
Primary myeofibrosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How can lymphoid haematological malignancies be subclassified?

A

Precursor cell malignancy

Mature cell malignancy

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

Give example of precursor lymphoid cell malignancy

A

Acute lymphoblastic leukaemia (B&T)

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

Give examples of mature cell lymphoid malignancy

A

Chronic lymphocytic leukaemia
Multiple myeloma
Lymphoid (Hodgkin & non Hodgkin)

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

What is the precursor to RBCs?

A

BFU-E

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

What myeloproliferative disorders are Ph negative (Philadelphia)?

A 
Polycythaemia vera (PV)
Essential thrombocythaemia (ET)
Primary myelofibrosis (PMF)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What MPD neoplasm is Ph positive?

A

Chronic myeloid leukaemia

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

Give examples of mutation mechanisms

A

DNA point mutations

Chromosomal translocations - creation of novel Fusion gene, disruption of proto-oncogene

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

Where are the mutations associated with Ph negative MPD?

A

Mutations in JAK2

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

What is JAK2?

A

Tyrosine Kinase signalling in haematopoesis

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

How does normal tyrosine kinases work?

A

Transmit cell growth signals from surface receptors to nucleus
Activated by transferring phosphate groups to self and downstream proteins
Normally held tightly in inactive state
Promote cell growth do not block maturation

19
Q

What are the components of MPD diagnosis?

A

Clinical features - symptoms, splenomegaly
FBC +/- bone marrow biopsy
Erythropoietin level (EPO)
Mutation testing

20
Q

How is polycythaemia vera usually diagnosed?

A

Incidental diagnosis on routine blood test

21
Q

What are the clinical symptoms of polycythaemia vera?

A

Headaches, light-headedness, stroke
Visual disturbances
Fatigue, dyspnoea
Aquagenic pruritis

22
Q

What is the aim of polycythaemia vera treatment?

A

Reduce risk of strokes
Target HCT <45%
Keep platelets below 400x10^9/l

23
Q

How is polycythemia vera treated?

A

Venesection (as if donating blood)
Cytoreductive therapy hydroxycarbamine
Aspirin (to prevent stroke)

24
Q

What is essential thrombocythaemia?

A

Chronic MPN mainly involving megakaryocytic lineage

Sustained thrombocytosis >600x10^9/L

25
Q

How is essential thrombocytopaenia usually diagnosed?

A

Incidental finding on FBC (50%)

26
Q

What symptoms/clinical signs may be present in essential thrombocytopenia?

A

Thrombosis: arteria or venous - CVA, gangrene, TIA, DVT, PE
Bleeding: mucous membrane and cutaneous
Headaches, dizziness, visual disturbances
Splenomegaly (modest)

27
Q

What is the treatment for essential thrombocytopenia?

A

Aspirin: to prevent thrombosis
Hydroxycarbamide: antimetaboilte, suppression of other cells as well
Anagrelide: specific inhibition of platelet formation, side effects include palpitations and flushing

28
Q

What is the prognosis of essential thrombocytopaenia?

A

Normal life span may not be changed in many patients
Leukaemic transformation in about 5% after >10 years
Myelofibrosis also uncommon, unless there is fibrosis at the beginning

29
Q

What is primary myelofibrosis?

A

A clonal myeloproliferative disease associated with reactive bone marrow fibrosis

30
Q

What may the presentation of primary myelofibrosis be?

A

Incidental (30%)
Cytopenias: anaemia or thrombocytopenia
Thrombocytosis
Splenomegaly: may be massice (Budd-Chiari syndrome)
Hepatomegaly
Hypermetabolic state: weight loss, fatigue, dyspnoea, night sweats, hyperuricaemia

31
Q

Why does primary myelofibrosis cause massive hepatosplenomegaly?

A

Bone marrow becomes fibrosed and scarred –> extramedullary haematopoeisis - bone marrow cell production moves to liver and spleen

32
Q

What are the blood film findings in primary myelofibrosis?

A

Leucoerythroblastic picture
Tear drop poikilocytes
Giant platelets
Circulating megakaryocytes

33
Q

What are the bone marrow findings in primary myelofibrosis?

A

Dry tap
Trephine - increased reticulin or collagen fibrosis, prominent megakaryocyte hyperplasia and clustering with abnormalities, new bone formation

34
Q

What is the prognosis of primary myelofibrosis?

A

Median 3-5 year survival but variable

35
Q

What are some bad prognostic signs in primary myelofibrosis?

A

Severe anaemia <100g/L
Thrombocytopenia <100x10^9/L
Massive splenomegaly

36
Q

What is the prognostic scoring system for primary myelofibrosis?

A

DIPPS
Score 0 = median survival 15 years
Score 4-6 - median survival 1.3 years

37
Q

What are the treatment options for primary myelofibrosis?

A

Supportive - RBC and platelet transfusion often ineffective because of splenomegaly
Cytoreductive therapy - hydroxycarbamide (for thrombosis, may worsen anaemia)
Ruxolotinib: JAK2 inhibitor (high prognostic score)
Allogenic SCT (potentially curative, reserved for high risk eligible cases)
Splenectomy for symptomatic relief: hazardous and often followed by worsening of condition

38
Q

What are the clinical symptoms/signs of chronic myeloid leukaemia?

A

Lethargy/hypermetabolism/thrombotic event: monocular blindness CVA, bruising, bleeding
Massive splenomegaly +/- hepatomegaly

39
Q

What are the haematological findings in chronic myeloid leukaemia?

A

Hb and platelets well preserved or raised
Massive leucocytosis 50-200x10^9/L
Blood films: neutrophils and myelocytes, basophilia

40
Q

Chronic myeloid leukaemia blood film

A

Leucocytosis between 50 - 500x109/l
Mature myeloid cells
Bi phasic peak Neutrophils and myelocytes
Basophils
No excess (<5%) myeloblasts
Platelet count raised/upper normal (contrast acute leuk)

41
Q

Diagnosing and monitoring response in chronic myeloid leukaemia

A

FBC and measure leucocyte count
Cytogenetics and detection of Philadelphia chromosome
RT-PCR of BCR-ABL fusion transcript which can be quantified by RQ-PCR to determine response to therapy

42
Q

What are the phases of CML?

A 
Chronic phase (median 3-4 years)
Advanced phases: accelerated phase (10-19% blasts, median duration 6-12 motnhs); blast crisis (>20% blasts, median survival 3-6 months)
43
Q

What treatment is available for CML?

A

Abl Tyrosine Kinase inhibitors (e.g. Imatinib)

44
Q

How do we monitor response to CML treatment?

A

Haematological response - WBC <10x10^9/L
Cytogenic response - partial 1-35% Ph positive, complete 0% Ph positive
Molecular (reduction in %BCT-ABL transcripts)

Haematology (E) (14 decks)

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