Brainscape's Knowledge GenomeTM

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


See full index

Haematology and Immunology > Myeloid Malignancy > Flashcards

Myeloid Malignancy Flashcards

1
Q

What are the Myeloid Malignancies?

A

Acute myeloid Leukaemia (AML)
Chronic Myeloid Leukaemia (CML)
Myelodysplastic syndromes (MDS)
Myeloproliferative neoplasms (MPN)

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

Pathology of AML

A

Leukemic cells do not differentiate

Bone marrow failure

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

Prognosis of AML

A

Rapidly fatal if untreated

Potentially curable

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

Subgroups of acute leukaemia

A

Acute myeloblastic leukaemia (AML)

Acute lymphoblastic leukaemia (ALL)

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

Presentation of Acute myeloblastic leukaemia

A

Bone marrow failure
- anaemia
- thrombocytopenic bleeding (purpura and mucosal)
- infection because of neutropenia (predominately bacterial and fungal)
Splenomegaly
Bone pain

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

Investigations of acute myeloblastic leukaemia

A

Blood count and blood film
Bone marrow aspirate / trephine
Cytogenetics (karyotype) from leukaemic blasts
Immunophenotyping of leukaemic blasts
CSF exam if symptoms
Targeted molecular genetics for associated gene mutations e.g. FLT3

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

Treatment of acute myeloblastic leukaemia

A

Supportive
Anti-leukaemic chemotherapy
- daunorubicin and cytosine arabinoside
Allogenic stem cell transplantation
All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) in low risk acute promyelocytic anaemia
Targeted treatment - midostaurin in FLT3 mutated AML

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

Pathology of CML

A

Leukaemic cells retain ability to differentiate

Proliferation without bone marrow failure

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

Prognosis of CML

A

Survival for a few years historically

Long term survival / possible cures with modern therapy

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

Presentation of CML

A 
Often no symptoms 
Low grade fever / sweating 
Malaise 
Anaemia
Splenomegaly, often massive - left upper quadrant fullness / leading to abdominal discomfort  
Weight loss
Hyperleukostasis 
- fundal haemorrhage and venous congestion 
- altered consciousness 
- resp failure
Gout
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

CML blood features

A

High WCC (can be VERY high)
Low Hb
High Platelets or may be reduced due to big spleen
High neutrophils
Anaemia
Blood film shows all stages of white cell differentiation with increased basophils
Bone marrow hypercellular
Increased mature granulocytes (basophils, eosinophils, neutrophils) so there is no immunosuppression
High or low red cell count due to the big spleen
Decreased leukocyte alkaline phosphatase

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

Treatment of CML

A

Tyro kinase inhibitors (TKIs)
- Imatinib - 1st line
Allogenic transplantation (few now) only in TKI failures
Bone marrow transplant only possible cure
Hydroxyurea
Interferon alpha

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

What are myelodysplastic syndromes?

A

Acquired clonal disorders of the bone marrow

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

What age are myelodysplastic syndromes common?

A

Elderly

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

Presentation of myelodysplastic syndromes

A

Macrocytic anaemia
Pancytopenia
Neutropenia
Bone marrow often hypercellular but the cells are not released into the blood

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

What is myelodysplastic syndromes in relation to leukaemia?

A

Pre-leukaemic

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

Prognosis of myelodysplastic syndromes

A

Pre-leukaemic

Fatal as result of progression to bone marrow failure or AML

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

Treatment of myelodysplastic syndromes

A

Supportive

Stem cell transplantation for the few younger patients

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

Types of myeloproliferative neoplasms (MPN)

A 
Polycythaemia Vera (PV)
Essential thrombocythemia (ET)
Idiopathic myelofibrosis (IM)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Mutations in myeloproliferative neoplasms

A

JAK2V617F mutation in > 98% of PV
PV in 50% of ET and myelofibrosis
CALR mutation in 25% of ET

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

Presentation of Polycythaemia Vera

A 
Headaches
Itch 
- intense
- after exposure to hot water or hot and humid weather 
Vascular occlusion 
Thrombosis (e.g. DVT)
TIA, stroke
Splenomegaly 
20% will suffer from gout
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Lab features of polycythaemia vera

A

Elevated red cells
Elevated platelets in 50%
Raised Hb and haematocrit
Tendency to have raised WCC and platelet count
A raised uric acid
True increase in red cell mass when blood volume measured

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Treatment of PV

A

Venesection to keep the haematocrit level below 0.45 in men and 0.43 in women
Aspirin
Hydroxcarbamide (HC)
Ruxolitnib (JAK2 inhibitor) in HC failures with systemic symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Prognosis of PV

A

Stroke and other arterial or venous thromboses if poorly controlled
Bone marrow failure from development of secondary myelofibrosis
Transformation to AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is Essential thrombocythaemia (ET)?

A

Myeloproliferative disease with predominant feature of raised platelet count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Mutations of ET

A

50% +ve for JAK2V617F mutation

25% positive for calreticulin (CALR) mutuation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Presentation of ET

A 
Symptoms of arterial and venous thrombosis 
Digital ischaemia 
Gout 
Headache 
Mild splenomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Treatment of ET

A

Aspirin

Hydroxycarbamide or anagrelide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What can ET progress to?

A

Myelofibrosis

AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What mutation causes > 98% of primary polycythaemia?

A

JAK2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Causes of haematological malignancy

A 
Radiation 
Non therapeutic
Chemo 
Smoking 
Chemicals e.g. benzene 
HIV 
HTLV1 and EBV 
Immunosuppressants 
Downs syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Who is AML most common in?

A

Elderly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is prognosis related to in AML?

A

Classification and cytogenic changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

How is chemotherapy given in AML?

A 
Blocks 
intensive for 10 days 
2 - 3 week break 
intensive chemo for 8 days
2 - 3 week break 
intensive chemo for 8 days 
2 - 3 week break 
May have a 4th dose
35
Q

AML success in under 60 y/o

A

80% success with ‘good chromosomal genes’
50% success for no or neutral chromosomal changes
20% success with ‘bad chromosomes’

36
Q

When is bone marrow transplant given in AML/ALL?

A

Not in remission post induction chemotherapy
High relapse risk (as assessed by chromosomes)
Relapsed disease

37
Q

Can parents be a bone marrow donor?

A

No

38
Q

How much of siblings statistically match for bone marrow transplant?

A

25%

39
Q

Why is rejection unlikely in bone marrow transplant?

A

Patients immune system has been ‘killed’ by the conditioning chemo +/- radiotherapy and will be replaced by the donors immune system

40
Q

Why do recipients of bone marrow transplant need immunosuppression?

A

To reduce the risk of graft versus host disease

41
Q

What is graft vs host disease?

A

New immune system from the donors bone marrow rejects recipients own tissue

42
Q

Who does CML effect?

A

More common in adults but effects everyone

43
Q

What chromosomal abnormality is seen in CML?

A

Philadelphia chromosome - t(9;22)

44
Q

The Philadelphia chromosome has a translocation between which chromosomes?

A

9 and 22

45
Q

What does the Philadelphia chromosome changes produce?

A

2 oncogenes BCR and ABL brought together to form

A new RNA sequence - creating tyrosine kinase

46
Q

What transcript is active in creating tyrosine kinase?

A

BCR-ABL

47
Q

Effects of BCR-ABL

A

Creates a new RNA sequence - tyrosine kinase

Activates proteins that speed up the cell cycle and inhibit DNA repair, increasing the risk of other mutations

48
Q

What can CML transform into?

A

AML (80%)

ALL(20%)

49
Q

If CML does transform into an acute leukaemia, what is the prognosis?

A

poor - 3 months

50
Q

How many blast cells in the marrow are normal?

A

5%

51
Q

How many blasts in the bone marrow is classed as leukaemia?

A

> 20%

52
Q

How many blasts in the bone marrow are classed as myelodysplastic syndrome?

A

5 - 19%

53
Q

What can myelodysplastic syndromes progress to?

A

Acute myeloid leukaemia

54
Q

What questions should be asked relating to tiredness?

A

How is it affecting life?
All the time?
Any reason for not sleeping?

55
Q

What features of feeling an abdominal mass would make you think that is was splenomegaly?

A

Can’t get above it
Moves with inspiration
May feel a splenic notch

56
Q

What is a feature of a mass that would indicate it was a kidney mass?

A

Ballotable

57
Q

What can be elevated in people with lymphomas?

A

LDH

58
Q

What level of melanocytes are usually in the peripheral blood?

A

None

59
Q

What type of cells are found in acute leukaemia?

A

Immature cells

60
Q

How fast does acute leukaemia come on?

A

Quickly - weeks - a month

61
Q

What blood result is often found to be VERY high in both CML and CLL?

A

WCC

62
Q

What test should be done usually if blood results are abnormal?

A

Bone marrow biopsy

63
Q

Would a bone barrow biopsy be done if for example the bloods weren’t effected in e.g. lymphoma?

A

No

64
Q

IMPORTANT INVESTIGATION I ALWAYS FORGET

A

Blood film

65
Q

What is cytogenetics used to analyse?

A

Chromosomes

66
Q

What is FISH?

A

Florescent marker attached to chromosome

67
Q

What is the 9 marker in CML when looking at FISH?

A

BCR

68
Q

What is the 11 marker in CML when looking at FISH?

A

ABL

69
Q

How do you test for the Philadelphia chromosome?

A

PCR
Cytogenetics
FISH

70
Q

What levels are tracked in CML to monitor treatment?

A

BRC-ABL

71
Q

Pathology of blast crisis in CML

A

Chronic phase -> accelerated phase -> AML

72
Q

What are the cancers that spread to bone?

A 
Prostate
Lung
Breast
Renal 
Thyroid
73
Q

What test should be done if concerned about K+ levels?

A

ECG

74
Q

How to bring down high K levels?

A

Salbutamol
Calcium gluconate if very high
Insulin dextrose (insulin and glucose)

75
Q

How to bring down high Ca levels?

A

IV fluids to rehydrate

Biphosphonates

76
Q

What is the issue when using bisphosphonates?

A

They can worsen renal failure

77
Q

What is the most common form of leukaemia in adults?

A

AML

78
Q

Poor prognostic features for AML

A

> 60 yo
20% blasts after first round of chemo
Cytogenetics - deletion of chromosome 5 or 7

79
Q

What classification system is used for AML?

A

FAB - French American british

80
Q

Describe the FAB classification of AML

A 
M0 - undifferentiated
M1 - without maturation 
M2 - with granulocytic maturation 
M3 - acute promyelocytic 
M4 - Granulocytic and monocytic maturation 
M5 - monocytic
M6 - eyrthroleukaemia 
M7 - megakaryoblastic
81
Q

Describe acute promyelocytic leukaemia (M3)

A

Associated with t(15;17)
Fusion on PML and RAR-alpha genes
Presents in younger people as opposed to other types of AML - average is 25 y/0

82
Q

What is often seen at presentation of acute promyelocytic leukaemia?

A

DIC

Thrombocytopenia

83
Q

What are seen on myeloperoxidase stain in acute promyelocytic leukaemia?

A

Auer rods

84
Q

Prognosis of acute promyelocytic leukaemia

A

Good

Haematology and Immunology (18 decks)

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