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Haematology > Myeloproliferative neoplasms MPN > Flashcards

Myeloproliferative neoplasms MPN Flashcards

1
Q

what are myeloproliferative neoplasms

A

clonal haemopoeitic stem cell disorder resulting in increased production of 1 or more type of haemopoeitic cells

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2
Q

how can myeloproliferative neoplasms MPN be classified

A

BCR-ABL1 positive

BCR-ABL1 negative

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3
Q

what kind of MPN is BCR-ABL1 positive

A

Chronic myeloid leukaemia CML

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4
Q

what kinds of MPN are BCR-ABL1 negative

A

polycythaemia vera PV
essential thrombocythaemia ET
myelofibrosis

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5
Q

what lab and clinical features suggest a MPN

A 
high granulocyte count +-
high red cell count +-
high platelet count +-
eosinophilia/basophilia
splenomegaly 
thrombosis in an unusual place
WITH NO REACTIVE EXPLANATION
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6
Q

what is chronic myeloid leukaemia

A

proliferation of abnormal granulocytes and their precursors (sometimes other cell lineages too)

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7
Q

how can CML progress

A

chronic phase –> accelerated phase –> blast crisis

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8
Q

list clinical features of CML

A 
asymptomatic 
splenomegaly 
gout 
hypermetabolic 
priapism 
hyperleukocytosis
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9
Q

list laboratory features of CML

A

blood count:
normal/low Hb
leukocytosis with neutrophilia, myelocytes, eosinophilia, basophilia
thrombocytosis

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10
Q

what genetic abnormality is a hallmark of CML

A

philadelphia chromosome

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11
Q

what is the philadelphia chromosome

A

mutated chromosome 22

usually a translocation t(9;22)

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12
Q

what is the pathology as a result of the philadelphia chromosome in CML

A

results in new chimaeric gene: BCR-ABL1

the product of this is a tyrosine kinase which causes abnormal phosphorylation resulting in the features seen in CML

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13
Q

what treatment is used in CML

A

tyrosine kinase inhibitors

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14
Q

what are common clinical features of BCR-ABL1 negative MPN

A 
asymptomatic 
increased cell turnover: gout, weight loss, fatigue, sweats
splenomegaly 
marrow failure 
THROMBOSIS : arterial and venous 
erythromelalgia
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15
Q

what is polycythaemia vera PV

A

high Hb/Hct with erythrocytosis (making it a true increase in red cell mass) but can have increased production in other cell lineages

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16
Q

what are differential diagnoses for polycythaemia

A 
Secondary polycythaemia (hypoxia induced): high altitude, COPD, OSA, smoking, EPO tumour 
Pseudopolycythaemia: dehydration, diuretics, alcohol, obesity
17
Q

clinical features of PV

A

same as MPN plus
headache, blurred vision, fatigue
thrombosis
aquagenic pruritis

18
Q

what is aquagenic pruritis and in which condition is it seen

A

itchy skin after a shower

seen in PV

19
Q

investigations for PV

A 
History and examination  
Rule out differentials 
FBC
Blood film 
Genetics: JAK2 mutation
20
Q

what is the implication of a JAK2 mutation in BCR-ABL1 negative MPN

A

JAK2 is a kinase

mutation causes loss of auto-inhibition resulting in increased erythropoeisis in the absence of a ligand

21
Q

treatment of PV

A

venesection: Hct<0.45
aspirin
cytotoxic chemotherapy - hydroxycarbamide

22
Q

what is essential thrombocythaemia ET

A

uncontrolled production of abnormal platelets

23
Q

what are consequences of ET

A

thrombosis

bleeding (due to acquired vWD)

24
Q

clinical features of ET

A

same as MPN
vaso-occlusive complications
bleeding

25
Q

what must you rule out before diagnosing ET

A

reactive thrombocytosis

26
Q

causes of reactive thrombocytosis

A

blood loss
infection/inflammation
malignancy (LEGO+C)
iron deficiency

27
Q

iron deficiency can cause a thrombocytosis, true or false

A

true

28
Q

investigations for ET

A

rule out CML
genetic testing: JAK2, CALR, MPL
bone marrow

29
Q

treatment of ET

A

antiplatelets

cytotoxic redutive agents - hydroxycarbamide, IFNa

30
Q

what are the subtypes of myelofibrosis

A

idiopathic

post polycythaemia/ET

31
Q

features of myelofibrosis

A

marrow failure
bone marrow fibrosis
extramedullary haemoatopoeisis

32
Q

what are the clinical features of myelofibrosis

A 
same as MPN 
panyctopaenia
- anaemia: SOB, fatigue, pallor
- thrombocytopaenia: bleeding, petechiae
- neutropaenia: infection, fever
splenomegaly 
hypercatabolic
33
Q

investigations and findings for myelofibrosis

A 
FBC
blood film 
dry aspirate
fibrosis on trephine biopsy 
genetics: JAK2 > CALR > MPL > triple negative
34
Q

treatment of myelofibrosis

A

supportive: transfusions, antibiotics
allogenic stem cell transplant
splenectomy
JAK2 inhibitors

35
Q

what are the characteristic blood film features of myelofibrosis

A

leukoerythroblastic

tear drop shaped poikilocyte RBC

36
Q

what are causes of leukoerythroblastic blood films

A

reactive eg sepsis
marrow infiltration
myelofibrosis

37
Q

MPN is the most common cause of raised blood cell counts, true or false

A

FALSE

reactive causes of high blood counts are more common

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