Myeloid Malignancies Flashcards

1
Q

2 Subgroups of Acute Leukaemia

A

Acute Myeloblastic

Acute Lymphoblastic

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

Which myeloid malignancy is rapidly fatal but potentially curable?

A

Acute Myeloid Leukaemia

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

Which cells fail to differentiate in Acute Myleoid Leukaemia?

A

Myeloid progenitor cells

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

3 Clinical Features of AML

A

Bone Marrow Failure
Thromocytopenic bleeding
Infection

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

Bone Marrow Failure experienced in AML causes which condition?

A

Anaemia

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

Which types of infection are common in AML and why?

A

Bacterial and fungal

Neutropenia

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

Why does bone marrow failure occur?

A

No differentiation of progenitor cells

Blast cells occupy >20% of bone marrow - visible on aspirate sample

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

Features and sites of thrombocytopenic bleeding

A

Purpura and mucosal bleeding

Mucosal bleeding - rectal, urogenic, skin, heavy periods

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

Example of a systemic fungal infection common in AML

A

Aspergillus fumigatus

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

How do we investigate AML?

A
Blood count
Blood film
Bone marrow aspirate/trephine
Immunophenotyping of blasts
CSF Examination
Molecular genetics
NGS myeloid gene panel
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which aquired gene mutations are associated with AML?

A

FLT3, NPM1, IDH1 &2

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

Treatment of AML

A
Supportive for bone marrow failure
Chemotherapy
Allogeneic stem cell transplantation
All-Trans Retionoic Acid (ATRA) & Arsenic Trioxide (ATO)
Targeted treatment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Example of chemotherapy used in AML

A

Daunorubicin and cytosine arabinoside (DA)
Gemtuzumab
CPX 351

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

Mechanism of gemtuzumab

A

Antibody with chemo attached

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

Mechanism of CPX 351

A

Delivers DA in micelles

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

In which specific condition is ATRA and ATO used?

A

Acute Promyelocytic Leukaemia

Bleeding out before treatment a big issue

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

Example of targeted treatment in AML

A

Midostaurin where FLT3 mutation identified

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

Features of Chronic Myeloid leukaemia

A
Anaemia
Splenomegaly
Weight loss
Hyperleukostasis
Gout
19
Q

Lab results in CML

A

WBC high
Platelets High
Anaemia

20
Q

Results on blood film in CML

A

Shows cells at all levels of differentiation

Basophil number high

21
Q

Describe the state of bone marrow cellularity in CML

A

Hypercellular

22
Q

Which genetic translocation occurs in the bone marrow and blood cells in CML

A

Philadelphia chromosomal translocation t(9;22)

23
Q

Treatment of CML

A

Tyrosine Kinase Inhibitors TKI

Allogeneic Stem cell transplantation - only if TKI fail

24
Q

Mechanism of TKI

A

Directly inhibit BCR-ABL

25
Q

Examples of TKI

A

Imatinib (Glivec)
Dasatinib
Nilotinib

26
Q

Name 3 Myeloproliferative Neoplasms

A

Polycythaemia Vera
Essential Thrombocythaemia (ET)
Idiopathic Myelofibrosis

27
Q

Features of blood results in Polycythaemia Vera

A

High red cells
High white cells
High platelets

28
Q

Polycythaemia Vera can occur secondary to…

A

Chronic Hypoxia

29
Q

Which mutation is common to all 3 MPNs?

A

JAK2 V617F

95% PV
50% ET &Myelofibrosis

30
Q

Which 2 mutations can occur in ET?

A

JAK2 V617F

CALR

31
Q

JAK2 is autophosphorylated upon the binding of which molecule?

A

EPO

32
Q

Autophosphorylation of JAK2 leads to downstream signalling to promote which process?

A

Red cell production

33
Q

What happens when JAK2 is mutated in MPN?

A

JAK2 is autophosphorylated even in absence of EPO

Leads to Myeloproliferative red cell production

34
Q

Clinical features of Polycythaemia vera

A
Headache 
Itch - aquagenic pruritus
Vascular occlusion
Thrombosis
TIA
Stroke
Splenomegaly
35
Q

Lab results in Polycythaemia Vera

A

High haematocrit
Raised white cell and platelet count
Raised uric acid
Measure blood volume to see true increase in red cell mass

36
Q

Treatment of Polycythaemia vera

A

Venesection
Aspirin
Hydroxycarbamide
Ruxolitinib (JAK2 Inhibitor) - only if hydroxycarbamide fails and exhibiting systemic symptoms

37
Q

High haematocrit causes what clinical symptom/sign

A

Sluggish circulation

38
Q

Poor management/control of PV can lead to..

A

Thrombosis and stroke
Bone marrow failure
Transformation to AML

39
Q

PV can be a precursor to which condition if not treated successfully?

A

AML

40
Q

Sign of essential thrombocytopenia

A

Raised platelet count

41
Q

Which genetic mutations are present in ET

A

JAK2 in 50%

CALR 25%

42
Q

Symptoms of ET

A

Arterial and venous thrombosis

Digital ischaemia- erythromyalgia, vascular occlusion

43
Q

Treatment of ET

A

Aspirin
Hydroxycarbamide
Anagrelide

44
Q

Signs of ET on blood film

A

Marked thrombocytosis with giant platelets