Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

3 Haematology > Chronic Myeloproliferative Disoders > Flashcards

Chronic Myeloproliferative Disoders Flashcards

(55 cards)

Start Studying
1
Q

myeloproliferative disorders tend to produce cells that are mature/immature

A

MATURE

a disease of too many cells

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

a hypercellular marrow will not contain mature cells T or F

A

F, can have mature cells eg in MPD

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

pathophysiology of MPD

A

too many mature myeloid cells

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

BCR-ABL1 positive disorder…

A

CML

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

name the BCR-ABL1 negative MPDs

A

idiopathic myelofibrosis
essential thrombocythaemia
polycythaemia rubra vera

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

which leukaemia is associated with the philiadelphia chromosome

A

CML

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

describe the blood count in MPD

A

high cell counts eg granulocytes, red cells, platelets

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

1st thing to do if you get an abnormal blood count

A

repeat

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

non-cancer causes of high Hb

A

chronic hypoxia eg in COPD/sleep apnoea

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

upper age limit of normal for a stem cell transplant

A

60-65

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

name the 3 phases of CML

A

chronic phase
accelerated phase
blast crisis (appears like acute leukaemia)

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

clinical features of CML

A 
asymptomatic
splenomegaly
hypermetabolic problems
gout
small vessel problems
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

why can you get gout in CML

A

high cell turnover causes inc breakdown deposits which cause gout

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

describe the blood count in CML

A

high WCC esp baso/eosinophils

normal-low Hb

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

what can be the earliest sign on a blood count of CML?

A

high platelets (thrombocytosis)

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

name the chromosomal translocation in philadelphia chromosome mutation in CML

A

t(9:22)

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

name the gene formed by the philadelphia chromosome

A

BCR-ABL1

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

name the gene product in CML and what it does

A

tyrosine kinase

abnormal phosphorylation

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

how many mutations are there in CML?

A

just 1 which is why its great for targeted therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

MPD tends to be symptomatic T or F

A

F, asymptomatic

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

erythromyalgia is a cutaneous sign of what?

A

thrombocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

symptoms of inc cell turnover

A

gout
fatigue
weight loss
sweats

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

symptoms of MPD

Study These Flashcards
A 
asymptomatic mostly
gout, fatigue, w loss, sweats
splenomegaly
marrow failure symptoms
thrombosis eg MI/erythromyalgia
24
Q

define PRV

Study These Flashcards
A

high Hb/haematocrit accompanied by erythrocytosis

25
difference between PRV and secondary polycythaemia
in secondary polycythaemia it is usually caused by chronic hypoxia, smoking, EPO secreting tumour
26
difference between PRV and pseudopolycythaemia
in pseudopolycythaemia you are dehydrated, on diuretics or are obese
27
what is the problem in pseudopolycythaemia
reduced plasma vol means haematocrit is raised when red cell count is normal
28
clinical features of PRV
ITCH esp in water MPD-like symptoms eg w loss, sweats, splenomegaly, marrow failure headache fatigue
29
pathophysiology of PRV
mutation in JAK2 gene causing loss of autoinhibition (activates erythropoeisis)
30
Ix PRV
FBC film mutational analysis for JAK2 mutation CXR, ABG, O2 sats for secondary causes
31
what kind of substance is JAK2
a kinase
32
how sensitive is JAK2 to PRV
v specific (95%)
33
Tx PRV
venesect to get haematocrit to <0.45 aspirin cytotoxic oral chemo via hydroxycarbamide lifestyle changes for thrombotic risk
34
how does the morphology of platelets differ from normal in ET?
much bigger than usual
35
define essential thrombocythaemia
uncontrolled production of abnormal platelets
36
symptoms of ET
thrombosis bleeding (can bind VWF and remove it from plasma) MPD like symptoms
37
Ix ET
exclude reactive thrombocytosis and CML mutation analysis bone marrow biopsy only if mutational analyses are negative
38
causes of reactive thrombocytosis
blood loss inflammation malignancy iron deficiency
39
appearance of ET on blood film
clustering of megakaryocytes | big megakaryocytes
40
what mutations can be present in ET
JAK2 calreticulin (CALR) MPL
41
Tx ET
antiplatelet eg aspirin | cytoreductive therapy eg hydroxycarbamide
42
causes of myelofibrosis
idiopathic | progressed from PRV or ET
43
teardrop shaped RBCs in peripheral blood...
idiopathic myelofibrosis
44
describe the blood film of idiopathic myelofibrosis
leukoerythroblastic (this is important!)
45
clinical features of MF
marrow failure splenomegaly hypercatabolism MPD like features
46
what happens in myelofibrosis
marrow failure and fibrosis | haematopoeisis is in liver and spleen as marrow is too damaged
47
Ix MF
blood film trephine marrow biopsy mutational analysis
48
causes of a leukoerythroblastic film
reactive eg sepsis/burns marrow infiltration eg malignancy spread to bone marrow myelofibrosis
49
what has to be present for a blood film to be leukoerythroblastic
nucleated red cells (erythroblasts) | myelocytes
50
Tx MF
supportive eg transfusion, antibiotics allogeneic stem cell transplant if fit JAK2 inhibitors
51
which BCR-ABL1 MPD has the worst prognosis?
MF
52
reactive causes of high granulocytes
infection | physiological eg surgery or steroids
53
reactive causes of high platelets
infetion iron deficiency malignancy blood loss
54
reactive causes of high red cells
dehydration eg diuretics/low intake | hypoxia induced eg COPD/sleep apnoea
55
what do reactive lymphocytes look like on blood film
blue cytoplasm | big nucleus

3 Haematology (30 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions