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Cancers of Myeloid Cell Origin Flashcards

1
Q

What is CML

A

Proliferation and differentiation of leukaemic stem cells
Increased end cells / monocytosis
Most commonly neutrophil affected
Present without bone marrow failure as no blocked differentiation
Proliferation of bone marrow that makes mature end-cells so lots of granulocytes

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2
Q

What is prognosis

A

Survival for a few years
Long term now achievable with modern therapy - attacking Philadelphia chromosome
CLL a bit behind

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3
Q

What is +Ve in majority of CML and what is important

A

Philadelphia chromosome t(9:22)(q34:q11)
Translocation 9-22
Give rise to mutant BCL-ABL on chromosome creating tyrosine kinase
Leads to uncontrolled cell cycling
Genetically unique as only require this one mutation to get disease rather than multiple hits
More +Ve prognosis in CML.
Dx by FISH / molecular testing

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4
Q

How does CML present

A

Insidious
WCC increasing = leucocytosis
Classicaly neutrophils + basophils
Can sometimes get thrombocytosis
Anaemia chronic disease - lethargy
Slight bone marrow failure (no neutropenia or thrombocytopenia)
Massive splenomegaly
B symptoms - Weight loss /fever / night sweats due to hyper metabolism
Anorexia due to spleen compressing stomach
Hyperleukostasis
Gout as high cell turnover
Increased viscosity due to increased cells
Have granulocytes at different stages

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5
Q

What do they not have

A

Neutropenia - raised

Thrombocytopenia - can get thrombocytosis

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6
Q

What causes weight loss

A

Hypermetabolic state

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7
Q

What does hyperleukostasis cause

A

Fundal haemorrhage
Venous congestion
Altered consciousness
Resp failure

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8
Q

What is important not to do due to increased viscosity

A

DO NOT TRANSFUSE

Anaemia is the only thing that is keeping viscosity at bay

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9
Q

What are lab features of CML

A

High WCC - extremely
Variable platelet - can be high due to inflammation
Whole spectrum of myeloid cells at different stages of maturation
Urate and B12 increased
Anaemia

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10
Q

What does blood film show

A

Increased cells and different types of white cells at different stages

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11
Q

What will bone marrow and blood cells contain

A

Philadelphia chromosome

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12
Q

What is diagnostic

A

Bloods
Blood film -
Philadelphia +Ve
Bone marrow = diagnostic

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13
Q

What is 1st line Rx in CML

A

Tyrosine kinase inhibitor (direct inhibitor of BCR-ABL on Philadelphia chromosome)
Known as Imaticinib
Normal life expectancy if maintain remission
Either take tablet for years or life

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14
Q

Other Rx options

A

Interferon alpha
Allogenic stem cell transplant - do this if relapse
Chemo NOT effective as can never get rid of all cells (used to be only option so used to be incurable)

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15
Q

What is most common leukaemia in adults

A

AML

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16
Q

How does it occur

A 
Primary
- Down's
- RT
' Chemo 
- Fanconi
Secondary transformation of myeloproliferative disorder
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17
Q

What happens in AML

A

Leukaemic cells do not differnetiate
Just proliferate
Leads to bone marrow failure
Rapid aggressive and fatal

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18
Q

What are features of AML

A 
Bone marrow failure 
Infiltration - HSM 
Bone pain
Skin involvement 
CNS = rare
Usually elderly
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19
Q

What suggests poor prognosis

A

> 60
20% blasts after chemo
Do not differentiate into different cell lines
Certain cytogenetics
APML t(15,17) can present in medical emergency
- DIC
- Haemorrhage
- Continuous therapy needed in these patients

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20
Q

How do you Dx / essential investigation

A 
Blood count + film
Bone marrow aspiration = diagnostic 
Cytogenetics/ immunophenotype to guide Rx 
CSF - more in ALL as CSF spread 
Targeted genetics
Increased use of NGS myeloid gene panels
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21
Q

What will blood count and film show

A 
AUER RODS 
WCC - high or low (usually high) 
Neutropenia
Thrombocytopenia
Anaemia 
Circulating leukaemic cells
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22
Q

What confirms Dx

A

Bone marrow aspirate
Blasts >20% = Dx
If 15% = myelodysplasia

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23
Q

If infiltrate CNS what do you get

A

Headache
CN palsy
More common in ALL

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24
Q

What genes and what do they suggest about prognosis

A 
FLT3 = worse but drugs to target
NPM1 = better
IDH1 = drugs to target
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25
How do you treat
Supportive Chemotherapy Allogenic stem cell to consolidate remission and cure
26
What is supportive care
Blood transfusion Platelet support Suport neutropenic sepsis risk
27
What is remission
Blood count returned to normal state | Blasts <5%
28
What are new treatments
``` ATRA - All trans retinoic acid - Continuous therapy against t(15,17) of RAR gene - APML type of AML ATO Targeted Ab against cell receptors New delivery systems ```
29
What is myelodysplastic syndromes
Acquired clonal disorders of bone marrow common in elderly Bone marrow trying to produce mature cells but never quite make it to maturity Blasts >15% but less than <20% Pre-leukaemic RF = smoking / previous chemo or RT
30
How does it present
Classically present with low blood count / cytopenia in peripheral blood but busy bone marrow full of pre-cursor ``` Bone marrow failure Macrocytic anaemia Pancytopenia HSM Will progress to AML ```
31
How do you Rx
Supportive - transfusion Few effective therapies Chemo Stem cell transplant in the younger
32
What are myeloproliferative disorders
Stem cell disorder that results in certain type of end cell / mature cell
33
What is linked to myeloproliferative disorders and what do they have potential to do
JAK2 mutation as means can produce cells e.g. red cells without EPO Turns of the stop button so just over proliferative Now used to Dx and patients no longer require bone marrow aspirate Pre-malignant Transform to AML
34
What are types of myeloproliferative disroder
Polycythermia vera - red cell Essential thrombocythaemia - platelet Idiopathic myelofibrosis - haemopoetic stem cell CML - WBC
35
What is polycythaemia vera
Clonal proliferation of marrow stem cell leading to excess red cells Often neutrophils and platelet increased as well
36
How does it present
``` Itch and headache = very common Raised Hb Hyperviscosity Headache Plethroic appearance Itch Painful hands and feet Vascular occlusion / thrombosis Splenomegaly causing feeling. of abdominal fullness Gout Low ESR Hypertension Haemorrhage ```
37
What is big risk
TIA / stroke
38
What causes haemorrhage
Abnormal platelets
39
How do you investigate
FBC / film U+E, LFT, ferritin, urate, LDH JAK2 +ve Cytogenetics to differentiate from CML
40
What does FBC show
Raised Hb + haematocrit Tend to have raised WCC / platelet MCV may be low as iron deficiency + low ferritin
41
What should you look for if raised Hb
Other causes Low sats Smoker COPD / lung disease
42
What is standard Rx
Venesection + aspirin
43
What is aim
To keep haematocrit below 0.45 in M and 0.43 in F | To prevent stroke and MI
44
What are other options
Hydroxycarbamide - suppress platelet | Ruxolitinimb - JAK2 inhibitor
45
What are complications
Stroke Bone marrow failure if secondary myelofibrosis occurs Transformation to AML
46
What are other causes of polycythaemia (cause same Sx)
COPD / lung disease / low sats - look for this Altitiude OSA Excessive erythropoetin - can be caused by renal tumour Dehydration / alcohol / diuretics causes relative
47
How do you differentiate between true polycythaemia and relative
Red cell mass studies - will be high if true
48
If JAK2 -ve and no obvious 2 cause for polycythameia what should you do
``` ABG ABdo USS Serum eryhropoetin Bone marrow aspiration Cytogenetics ```
49
What is essential thrombocythaemia
Raised platelets
50
What mutation
JAK2 | CARL
51
What symptoms
``` Arterial and venous thrombosis Digital ischaemia Gout Mild splenomegaly Normal ferritin ```
52
How do you Rx
Manage vascular RF | Aspirin + hydroxycarbamide to get platelet down as high risk of thrombosis
53
What can it progress too
Secondary myelofibrosis | AML
54
What is idiopathic myelofibrosis
Fibrosis of bone marrow due to hyperplasia of abnormal megakaryocytic / cell lines Cytokines cause release of platelet derived growth factor and stimulation of fibroblast Can be primary or secondary to polycythaemia or essential thrombocythaemia
55
What does this lead too
Haemotopoeisis developing in liver and spleen as can't occur in marrow Can cause HSM + portal hypertension
56
How does it present
``` Bone marrow failure - Anaemia - Low WCC Elderly + extreme fatigue Massive splenomegaly Hypermetabolic - weight loss / night sweats Symptoms of cytopenia Thrombotic events ```
57
What are lab results
``` Anaemia Can get high or low WCC depending on disease High or low platelet High urate High LDH ```
58
What can you not too
Bone marrow biopsy as fibroses so just get dry tap | Trephine will be fibrotic and hyper cellular
59
What is seen on film
Tear drop poikilocytes
60
How do you Rx
Transfusion + supportive Hydroxycarbamide Ritoxicilinum - JAK2 inhibitor Allogenic stem cell transplant = only curative
61
How do you assess raised Hb / haematocrit
``` History Examination Bloods JAK2 USS to look for splenomegaly EPO Red cell mass studies Bone marrow = rarely done ```
62
What is important in the Hx
Any reasons for polycythaemia e.g. smoking Any symptoms suggestive of polycythamia vera Assess thrombotic risk
63
What do you look for in examination
Splenomegaly Chest signs O2 sats
64
What bloods
Repeat FBC, U+E, LFT, urate, LDH, ferritin
65
What confirms myelproliferative disorder
+ve JAK2 mutations
66
What does EPO tell you
If low = primary polycythaemia Normal = unhelpful If high = secondary
67
What are red cell mass studies useful for
Work out if true or apparent polycythaemia
68
What causes primary thrombocytosis
``` Overproduction in bone marrow usually due to myeloproliferative disorder Essential thrombocytosis Myelofibrosis Ppolycythaemia CML ```
69
What causes secondary
``` Systemic response to infection Inflammation - RA Tissue damage - post surgery Malignancy Iron or B12 deficiency ```
70
What could you ask in history
``` Infection Surgery Bleeding Weight loss Joint pain Pruritus ```
71
What do you look for in examination
Splenomegaly | Signs of malignancy / inflammation / infection
72
What investigations
``` FBC, film, U+E, LFT, urate, LDH CRP/. ESR Ferritin JAK2 USS/. CT ```
73
What can help differentiate
Primary patient usually well - Itch / thrombosis / splenomegaly often associated Secondary patient usually unwell
74
Background
OK
75
Where are myeloid precursor cells supposed to liver
In BM = only place
76
What happens if begins to grow out of control e.g AML / myelodysplasia
Very busy bone marrow Increasing cells in blood count as leak out Abnormal cells in BM expand in number and crowd out normal precursor BM starts to fail in ability to produce normal cells = cytopenia Abnormal cells leak out into blood and can see leukaemia cells
77
What can AML purely present with
Cytopenia | May just be neutropenic but will eventually get all
78
Overproliferation of immature cells
AML
79
Overproliferation of mature cells
ET
80
Similarity between myeloproliferative and myelodysplasia
Both myeloid Both slow moving Both oveproliferation of myeloid precursor
81
Difference
Myeloproliferative - Cells still normal enough to make it to maturity of whatever lineage - Leads to too many blood cells - Aim to get cell count down Myelodysplasitc - Cells proliferate but do not survive - BM can look similar as full of precursor but blood count low / cytopenia
82
How does CML overlap with both
Proliferation of WCC - neutrophilic / monocytosis Get loss of mature RBC and platelet formation Often a leucocytosis with anaemia / thrombocytopenia or both
83
What can result from myeloproliferative / dysplasia
Secondary AML Cells already have a genetic abnormality so more likely to transform More difficult to Rx if secondary as more mutations

Haematology (13 decks)

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