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3 Haematology > Chronic Myeloproliferative Disoders > Flashcards

Chronic Myeloproliferative Disoders Flashcards

1
Q

myeloproliferative disorders tend to produce cells that are mature/immature

A

MATURE

a disease of too many cells

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2
Q

a hypercellular marrow will not contain mature cells T or F

A

F, can have mature cells eg in MPD

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3
Q

pathophysiology of MPD

A

too many mature myeloid cells

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4
Q

BCR-ABL1 positive disorder…

A

CML

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5
Q

name the BCR-ABL1 negative MPDs

A

idiopathic myelofibrosis
essential thrombocythaemia
polycythaemia rubra vera

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6
Q

which leukaemia is associated with the philiadelphia chromosome

A

CML

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7
Q

describe the blood count in MPD

A

high cell counts eg granulocytes, red cells, platelets

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8
Q

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

A

repeat

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9
Q

non-cancer causes of high Hb

A

chronic hypoxia eg in COPD/sleep apnoea

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10
Q

upper age limit of normal for a stem cell transplant

A

60-65

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11
Q

name the 3 phases of CML

A

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

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12
Q

clinical features of CML

A 
asymptomatic
splenomegaly
hypermetabolic problems
gout
small vessel problems
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13
Q

why can you get gout in CML

A

high cell turnover causes inc breakdown deposits which cause gout

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14
Q

describe the blood count in CML

A

high WCC esp baso/eosinophils

normal-low Hb

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15
Q

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

A

high platelets (thrombocytosis)

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16
Q

name the chromosomal translocation in philadelphia chromosome mutation in CML

A

t(9:22)

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17
Q

name the gene formed by the philadelphia chromosome

A

BCR-ABL1

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18
Q

name the gene product in CML and what it does

A

tyrosine kinase

abnormal phosphorylation

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19
Q

how many mutations are there in CML?

A

just 1 which is why its great for targeted therapy

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20
Q

MPD tends to be symptomatic T or F

A

F, asymptomatic

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21
Q

erythromyalgia is a cutaneous sign of what?

A

thrombocytosis

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22
Q

symptoms of inc cell turnover

A

gout
fatigue
weight loss
sweats

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23
Q

symptoms of MPD

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

define PRV

A

high Hb/haematocrit accompanied by erythrocytosis

25
Q

difference between PRV and secondary polycythaemia

A

in secondary polycythaemia it is usually caused by chronic hypoxia, smoking, EPO secreting tumour

26
Q

difference between PRV and pseudopolycythaemia

A

in pseudopolycythaemia you are dehydrated, on diuretics or are obese

27
Q

what is the problem in pseudopolycythaemia

A

reduced plasma vol means haematocrit is raised when red cell count is normal

28
Q

clinical features of PRV

A

ITCH esp in water
MPD-like symptoms eg w loss, sweats, splenomegaly, marrow failure
headache
fatigue

29
Q

pathophysiology of PRV

A

mutation in JAK2 gene causing loss of autoinhibition (activates erythropoeisis)

30
Q

Ix PRV

A

FBC
film
mutational analysis for JAK2 mutation
CXR, ABG, O2 sats for secondary causes

31
Q

what kind of substance is JAK2

A

a kinase

32
Q

how sensitive is JAK2 to PRV

A

v specific (95%)

33
Q

Tx PRV

A

venesect to get haematocrit to <0.45
aspirin
cytotoxic oral chemo via hydroxycarbamide
lifestyle changes for thrombotic risk

34
Q

how does the morphology of platelets differ from normal in ET?

A

much bigger than usual

35
Q

define essential thrombocythaemia

A

uncontrolled production of abnormal platelets

36
Q

symptoms of ET

A

thrombosis
bleeding (can bind VWF and remove it from plasma)
MPD like symptoms

37
Q

Ix ET

A

exclude reactive thrombocytosis and CML
mutation analysis
bone marrow biopsy only if mutational analyses are negative

38
Q

causes of reactive thrombocytosis

A

blood loss
inflammation
malignancy
iron deficiency

39
Q

appearance of ET on blood film

A

clustering of megakaryocytes

big megakaryocytes

40
Q

what mutations can be present in ET

A

JAK2
calreticulin (CALR)
MPL

41
Q

Tx ET

A

antiplatelet eg aspirin

cytoreductive therapy eg hydroxycarbamide

42
Q

causes of myelofibrosis

A

idiopathic

progressed from PRV or ET

43
Q

teardrop shaped RBCs in peripheral blood…

A

idiopathic myelofibrosis

44
Q

describe the blood film of idiopathic myelofibrosis

A

leukoerythroblastic (this is important!)

45
Q

clinical features of MF

A

marrow failure
splenomegaly
hypercatabolism
MPD like features

46
Q

what happens in myelofibrosis

A

marrow failure and fibrosis

haematopoeisis is in liver and spleen as marrow is too damaged

47
Q

Ix MF

A

blood film
trephine marrow biopsy
mutational analysis

48
Q

causes of a leukoerythroblastic film

A

reactive eg sepsis/burns
marrow infiltration eg malignancy spread to bone marrow
myelofibrosis

49
Q

what has to be present for a blood film to be leukoerythroblastic

A

nucleated red cells (erythroblasts)

myelocytes

50
Q

Tx MF

A

supportive eg transfusion, antibiotics
allogeneic stem cell transplant if fit
JAK2 inhibitors

51
Q

which BCR-ABL1 MPD has the worst prognosis?

A

MF

52
Q

reactive causes of high granulocytes

A

infection

physiological eg surgery or steroids

53
Q

reactive causes of high platelets

A

infetion
iron deficiency
malignancy
blood loss

54
Q

reactive causes of high red cells

A

dehydration eg diuretics/low intake

hypoxia induced eg COPD/sleep apnoea

55
Q

what do reactive lymphocytes look like on blood film

A

blue cytoplasm

big nucleus

3 Haematology (30 decks)

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