Brainscape's Knowledge GenomeTM

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


See full index

Year 5 Haematology > cml and myeloproliferative diseases > Flashcards

cml and myeloproliferative diseases Flashcards

1
Q

myeloid means

A

white blood cells from bone marrow

erythrocytes

megakaryerthrocyte > platelet

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

relative polycythaemia or true?

A

g/l haemoglobin

polycythaemia haemoglobin is raised

is it excess haemoglobin?
or is it plasma ? if it goes down

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

how can true polycythaemia be categorised?

A

secondary /no malignant

primary - myeloproloiferative

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

MPD examples?
Ph -

A

PV
ET
primary myelofibrosis (PMF)

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

PH +
myeloproliferalative

A

CML

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

raised cell mass and normal plasma volume means?

A

true polycythaemia

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

secondary polycythaemia ?
what would be raised?

A

EPO

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

why does erythropoeitin raise in hypoxic lung disease / cyanotic heart disease??

A

inadequate oxygenation of the blood

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

appropriate reasons for epo to be raised?

A

altitude
high affinity haemoglobin
hypoxic lungs
cyanotic heart

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

inappropirate raised epo?

A

renal disease?
cysts / tumours/ inflammation
uterine myoma
liver lung tumours

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

why does high altitude cause raised erythropoeitin?

A

it happens because of low atmospheric pressure at high altitudes. Oxygen saturation levels refer to the extent hemoglobin is bound or saturated to oxygen

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

myeloproliferative means?

A

excess of mature cells

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

what processes can be disrupted by DNA mutation?

A

cellular differentiation - type 2
cellular proliferation - type 1

anti-apotosis

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

mutation mechanisms?

A

dna point mutation
chromosomal translocations
creation of novel fusion gene
disruption of proto-oncogene

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

what is a disrupted proto-oncogene

A

this is something that normally controls the cell growth
but when a proto-oncogene is disrupted it causes accelerated and uncontrolled growth

a gene that promotes the specialization and division of normal cells that becomes an oncogene following mutation

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

tyrosine kinsae are normally

A

inactive

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

point mutation in _____ occurs in polycythaemia vera

how does it work?

A

JAK2
calreticulin
cMPL

always activated does not require binding

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

diagnosis of MPD

A

clinical features
splenomegaly

fbc/bone marrow biopsy
EPO
mutation test

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

PV epidemiology
incidence
mean age?
male/female?

A

2-3/100000

more males/females
mean age at diagnosis - 60

5% below <40

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

fbc ? PV

A

Hb 184g/l hct 0.55

21
Q

symptoms of hyper viscose blood?

A

headaches /light headedness
TIA
stroke
Visual Disturbances
fatigue/dyspnoea

aquagenic pruritus
peptic ulceration

test for JAK2 v617F

22
Q

what is principle of mx?
target?

A

reduce HCT
<45%
venesection
hydroxycarbamide

risk of thrombosis reduction
control hct
keep platelets below 400x109/l

23
Q

Essential thrombocytopenia is?

A

normal rbc
normal wbc

isolated elevation of platelets

24
Q

401x10^9/L platelet count?

A

either ET
or reactive

25
Q

if it is 1000000x10^9 platelet count?

A

defo ET

26
Q

ET clinical presentation
dysfunctional platelet

excess platelet

A

mucous membrane/ cutaneous

thrombosis /
CVA, gangrene TIA
DVT / PE

headaches/ dizzy/visual
splenomegaly

27
Q

ET mx?

A

prevent bleed/ thrombobosus

aspirin
hydroxycarbamide

anagrelide - specific inhibition of platelet formation S/E palpitation

28
Q

prognosis of Et

A

normal

leukaemic transfomration
myelofibrosis also uncommon

29
Q

PMF

what is it?

A

clonal (malignant abnormal clone)

malignant clone stimulates depositiion of fibrin and reticular tissue in bone marrow

reactive bone marrow fibrosis -
polyclonal fibrosis

= scar tissue in bone marrow

30
Q

presents with PMF?

A

cytopenia - anemia / thrmobocytopenia

thrombocytosis
splenomegaly
peripheral blood count : disease is in bone marrow

31
Q

PMF - splenomgelay

A

massive
iliac fossa palpable
hepatomgelay
as bone marrow becomes fibrotic - haemopoeisis moves to liver and spleen

so they expand
as bone marrow cells move there

hypermetabolic disease

32
Q

blood film of PMF

A

tear drop poikilocytes
giant platelets

33
Q

bone marrow finding in PMF

A

dry tap
as bone marrow is replaced by reticulin or collagen fibrosis

34
Q

bad prognostic signs of myelofibrosis

A

cytoreductive therapy would worsen the anemia / neutropenia

inhibitive drug - jak2 inhibiotr not that good

allogenic SCT - potentially curative but so dangerous

35
Q

primary myelofibrosis treatment 2

A

ruxolitinib
JAK2 inhibitor

allogenic SCT
high risk eligible cases reserved

splenectomy for symptomatic relief- worsens condition
splenomegaly is painful but they need erythropoeisis

36
Q

CML
philadelphia chromosome?

A

radiation exposure risk factor
acquired genetic change

37
Q

CML presents?

A

hypermetabolic
lethargy
thrombotic event
bruising
bleeding

38
Q

what thrombotic event can CML present with?

A

monocular blindness CVA
retinal artery vein

39
Q

exam findinga of cml

A

massive hepatomegaly but no lymphadenopathy is CML

40
Q

massive hepatomgelay with generalised lymphadenopathy is what ?

A

an indolent lymphoma

41
Q

FBC

leucocytosis ?

A

Hb and platelets well preserved / raised
leucocytosis 50-500 x 10^9/L

42
Q

blood film
CML

A

neutrophilia
meylocytes
basophilia

43
Q

biphasic peak?

A

most mature cells - granulocytes
normal in bone marrow not normal in peripherla

44
Q

t(9,22)

A

9 translocates to 22

45
Q

abl is a

A

tyrosine kinase- abl is overexpressed

bcr - housekeeping

46
Q

clinical course of CML

A

imatinib is the droooooog

47
Q

assessing response / monitor therapy

A

haematological response
cytogenic - 20 metaphases at diagnosis

most sensitive way is to look at BCR-ABL
>10%,1%,0.1%

48
Q

CML, myelofibrosis diseases cause massive

A

hepato-splenomegely with no lymphadenopathy

49
Q
A

Year 5 Haematology (10 decks)

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