Haematological Malignancies - Leukaemia Flashcards

1
Q

development of blood cancer

A
  • cell development is stopped in the BM where undeveloped cells divide uncontrollably, which build up in the BM and spill into the blood
  • genetic mutations cause cells to NOT fully develop into full RBC, which causes the cell to change its behaviour
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2
Q

where do GM occur

A

on the chromosome

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3
Q

what is the disease determined by

A

the stage at which the mutation occurred during cell development as well as the SC and the type

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4
Q

what is translocation

A

one part of the chromosome breaks off and attaches to another or exchanges places

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5
Q

what is deletion

A

segment of the chromosome is lost during replication

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6
Q

what is inversion

A

piece of the DNA on the chromosome gets inversed

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7
Q

what do genetic mutations influence

A

treatment options

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8
Q

male Hb levels

A

130-180g/l

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9
Q

female Hb levels

A

115-165g/l

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10
Q

WBC normal levels

A

4-11 x 10^9/l

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11
Q

platelet normal levels

A

250-500 x 10^9/l

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12
Q

symptoms of anaemia

A

breathlessness, fatigue, blueish skin

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13
Q

thrombocytopenia

A

excessive bleeding, purple tinges, repeated infections

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14
Q

stats of leukaemia

A

most common paediatric cancer
12th most common in UK
12th most common cancerous death
3% smoking
9% ionising radiation
lowest in asian and black groups

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15
Q

two types of leukaemia

A

chronic and acute

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16
Q

what is the most common leukaemia acute or chronic for paediatrics

A

acute

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17
Q

what determines what type of leukaemia it is

A

the B or T cell

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18
Q

what are the acute types

A

ALL = acute lymphoblastic leukaemia
AML = acute myeloid leukaemia

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19
Q

what are the chronic types

A

CLL = chronic lymphoblastic leukaemia
CML = chronic myeloid leukaemia

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20
Q

what does acute mean

A

sudden onset, 4-6 weeks
curable

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21
Q

what does chronic mean

A

long standing, gradual evolution, incurable

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22
Q

what are the risk factors for leukaemia

A

genetic disorders: down syndrome [15x AML, 30x ALL], faticoni anaemia, ataxi telangietasia, bloom syndrome
benzene exposure
previous chemo
previous RT [over-radiated bone at a young age]
medications to lower immunity for organ transplant
HIV
auto-immune disease
chernobyl

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23
Q

symptoms for leukaemia

A

bruising, bleeding easily
weightloss
flu-like symptoms
weak/tired
high temp
not able to shake off a cough or cold
looking pale/washed out
feeling full in tummy
breathlessness [anaemia]
pain in bone and joints
swollen LN

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24
Q

what is the acronym for symptoms

A

T = tiredness + exhaustion
E = excessive sweating
S = sore bones and joints
T = terrible bruising, unusual bleeding

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25
how are leukaemia's diagnosed
FBC: shows any non functional cells bone marrow aspiration US: enlargements within the lymphatic system or spleen, RT can control an enlarging spleen
26
staging of leukaemia
IT CAN'T BE STAGED
27
what is it called if it is found in the CNS
it has infiltrated, IT IS NOT MET SPREAD
28
what is leukaemia confirmed by
a chest x-ray (indicates SCVO formation), lumbar puncture takes CSF fluid which checks for CNS involvement
29
what is the survival for leukaemia
90%
30
when is a stem cell given
if there is more than 10% of leukaemia cells, which means high dose chemo + TBI will be given to dampen immune system
31
when is the rejection rate lower for SC transplants
after prior trt
32
what is an SVCO
compression of the SVC which reduces blood flow, causing the patient to bleed out, can be caused by leukaemia due to the T cells accumulating in the thymus [located in the mediastinum]
33
what is the ratio for ALL
50:50
34
how does ALL develop
over days/weeks where lymphocytes will build up rapidly and spill over into the blood
35
what age is most common for ALL
0-4
36
what happens if ALL is left untreated
pt will die within a few weeks or months
37
what are the risk factors of ALL
maternal infections during pregnancy delayed infections lack of exposure to infections as a child keeping things too clean, not exposed to natural pathogens RARE in children with allergies
38
what is the % of children with leukaemia have an ALL
80%
39
what does high toxicities in the TYA group cause
high chance of relapse
40
RT for ALL
testes relapse [T-cell]: ortho 24Gy in 12, penis is shielded to prevent the narrowing of the urethra TBI
41
if ALL involves the meninges what RT is given
whole brain field extends to c-spine [reeds baseline] CNS relapse: 24Gy in 15 + intrathecal chemo
42
what syndrome can be caused during chemo etc
tumour lysis syndrome effective treatment, why chemo is given gradually
43
risk factors of AML
autoimmune diseases: rheumatoid arthitius, autoimmune haemolytic anaemia, ulcerative colitis smoking: longer and quantity previous RT exposure to benzene prior breast treatments or NHL trt with certain chemo drugs: chloroambucil, melphalan, cyclophosphamide
44
where do AML arise from
SC or a myeloid blast cell
45
age of AML
>70 = 40% 80% = adults 20% = children
46
what does the treatment of leukaemia depend on
protocol at diagnosis and the drug combination
47
what treatment option is uncommon for leukaemia
watch and wait
48
what are the chemo stages
steroid pre-phase: steroids help body accept chemo, making it more efficient induction consolidation intensification maintenance intrathecal chemo: direct into CSF fluid if infilitrated into CNS
49
what leukaemia is not for maintenance chemo
AML
50
what are the chemo drugs for leukaemia
doxorubicin vincristine cyclophosphamide cytarabine asparaginase methotextrate
51
what are the consolidation drugs
cyclophosphamide cytarabine asparaginase methotrexate etoposide daunorubicin mercatopurine doxorubicin
52
what are the maintenance drugs
vincristine mercatopurine methotrexate predrusolone
53
why are maintenance drugs given
low dose up to 2 years keeps pt in remission however high chance of relapse in the first two years
54
what are the risk factors of chronic leukaemia
ionising radiation, most are sporadic
55
how are chronic leukaemia diagnosed
blood test: blast or mature incidental routine: FBC lumbar puncture bone marrow examination
56
what is the chronic myeloid philadelphia
rearrangement of chromosome 9 (ABL 1 gene present) + 22 (BCR gene) BCR-ABL1 gene increases tyrosine kinase which encourages leukaemia cells to be produced
57
what pathway does CML follow
TRIPHASIC
58
what is the triphasic pathway
chronic = high number of granulocytes, few blast cells (granulocytes collect in the spleen causing splenomegaly), usually 2-6 years. aim to keep people in this phase accelerated = evolving blastic = high number of blast cells, grown out of the BM, survival is 2-6 months
59
what is the chronic treatment
- TKI (imatinub, bosutinib, dasatinib, niolotinib) + allopuriol - stops enzyme action on cell cycle - remission up to 10 yrs - chemo can be given if severe TKI toxicity (fludarabine, idarubicin, cytarabine) - SC: allogenic with the aim to cure, it requires high dose chemo and TBI
60
what is the first line treatment for CML
TKI if can't tolerate then chemo if not this then SC
61
what is the accelerated trt
TKI (nilotinib = stronger but more SE) chemo, SC
62
what is the blast trt
intensive, high dose combination chemo SC is only given if chemo indices second chronic phase/ remission + pt is fit enough
63
what supportive therapies can be done for CML
remove excess WBC = neukapheresis surgery to remove blood clots, prevent blockages WBC removed, RBC, platelets returned to circulation RT for enlarged spleen
64
what are the SE for trt for CML
nausea vomiting diarrhoea constipation fluid retention lymphatic blockages loss of appetite muscle and bone pain skin changes [prurtis, rashes]
65
survival for CML
good TKI response = 90% tablet for the rest of their life
66
what are the risk factors for CLL
unknown no link to IR no link to infection
67
is presentation slow or fast for CLL
slow due to the WBC almost being fully developed, still working but not as good at fighting infection. immune response is only initiated when something is wrong with your cells
68
how do CLL present
accidental via FBC: slight anaemia, purpura, bruising is rare weightloss/ loss of appetite: compression of stomach enlarged spleen repeated infections lymphadenopathy: axillary, cervical, inguinal, painless, can be misdiagnosed as lymphoma due to enlarged LN infiltrate the skin: exfolliative dermatitis
69
what trt is common for CLL
watch and wait saves trt options for later on
70
what is stage A for a CLL
<3 areas of enlarged LH good
71
what is stage B for a CLL
3 or more areas of enlarged LN and high levels of WBC intermediate risk
72
what is stage C for CLL
enlarged LN or spleen, high levels of WBC evidence of anaemia and/or thrombocytopenia poor
73
what is trt for stage A of a CLL
no active trt, reg follow up, manage symptoms
74
what is trt for stage B and C of a CLL
chemo = chloroambucil or bendomustine [single] FC - fludarabine + cyclophosphamide [combo] steroids = lymphocytic [supportive] long term SE monoclonal antibodies = nituxumab (R) chemo-immuno = R-FC RT = enlarged nodes, spleen TBI can be given
75
is a SC given for a CLL
no as risks outweigh the benefits
76
what is done for an enlarged LN for RT due to a CLL
ortho single direct or A/P 10-20Gy in 5-10