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Haematology Year 3 > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

what is meant by myeloproliferative

A

abnormal proliferation of myeloma cells in the marrow

e.g. granulocytes, red cells + platelets

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2
Q

what are myeloproliferative disorders

A

clonal haemopoietic stem cell disorders with an increased production of one or more types of haemopoietic cells

maturation is preserved

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3
Q

how does acute leukaemia and myeloproliferative disorder differ on blood film

A

acute leukaemia = lots of primitive cells

MPD= numbers are increasing but normal maturation

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4
Q

subdivisions of chronic myeloproliferative disorder

A

BCR-ABL 1 negative

BCR- ABL 1 positive

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5
Q

conditions that are BCR-ABL 1 negative

A

myelofibrosis
essential thrombocytosis (over-production of platelets)
polycythaemia rubra vera (overproduction of red cells)

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6
Q

conditions that are BCR-ABL 1 positive

A

Chronic myeloid leukaemia

  • over production of granulocytes
  • philadelphia chromosome
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7
Q

what is essential thrombocytosis

A

over production of platelets

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8
Q

what is polycythaemia rub vera

A

over production of red cells

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9
Q

when should you consider a myeloproliferative disorder

A
  • high granulocyte count
  • high RBC count / Hb
  • high platelets count
  • eosinophilia/basophilia
  • splenomegaly
  • thrombosis in an unusual place
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10
Q

what is chronic myeloid leukaemia

A

proliferation of myeloid cells

  • granulocytes + their precursors (eosinophils, basophils, neutrophils)
  • other lineages (platelets)

fatal without stem cell/bone marrow transplant

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11
Q

pathology of chronic myeloid leukaemia

A

chronic phase with intact maturation 3-5 years

followed by ‘blast crisis’- similar to acute leukaemia with maturation defect

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12
Q

clinical features of chronic myeloid leukaemia

A 
asymptomatic 
splenomegaly
hyper metabolic symptoms 
gout
priaprism
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13
Q

what might people complain of in splenomegaly

A

feel hungry but get full very quickly when they start eating

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14
Q

lab findings in chronic myeloid leukaemia

A

normal/low Hb

leucocytosis with neutrophilia and myeloid precursors (myelocytes)
eosinophilia, basophilia

thrombocytosis (increased platelets)

+ve BCR- ABL gene
+ve philadelphia chromosome

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15
Q

hallmark of chronic myeloid leukaemia

A

philadelphia chromosome

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16
Q

what is a Philadelphia chromosome

A

part of one chromosome moving to another

chr 22 gives some info to chr 9

makes the BCR-ABL 1 gene

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17
Q

what does the Philadelphia chromosome result in

A

formation of new gene BCR-ABL 1

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18
Q

what does the BCR- ABL 1 gene cause

A

tyrosine kinase which causes abnormal phosphorylation (signalling) leading to haematological changes in chronic myeloid leukaemia

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19
Q

treatment of chronic myeloid leukaemia

A

Imatinib (tyrosine kinase inhibitor)

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20
Q

common features of myeloproliferative disorders

  • myelofibrosis
  • essential thrombocytosis
  • PRV
A

increased cellular turnover (gout, fatigue, weight loss, sweats)

splenomeglay

marrow failure

thrombosis
- arterial/venous including TIA, MI, claudication, erythromelalgia

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21
Q

what is erythromelalgia

A

thrombosis in hands + feet

22
Q

findings in polycythaemia rubra vera (PRV)

A

HIGH HB/haematocrit accompanied by erythrocytosis (a true increase in red cell mass)

23
Q

what is erythrocytosis

A

true increase in red cell mass

24
Q

what must polycythaemia rubra vera be distinguished from

A

secondary polycythaemia

pseudopolycythaemia

25
Q

causes of secondary polycythaemia

A

hypoxia

  • copd
  • smoking
  • altitude

Inappropriate EPO production
erythropoietin secreting tumour
- liver, renal, cerebellar

26
Q

pseudopolycythaemia

A 
decreased plasma volume therefore relative increase in concentration 
caused by:
dehydration
diuretic therapy 
obesity
27
Q

specific features of PRV

A

itch – aquagenic puritis - patient has a shower and then has an uncontrollable itch
splenomegaly (do not get with secondary/pseudo)
increased WBC + platelets (usually normal in secondary/pseudo)

28
Q

what is raised in PRV: blood viscosity or plasma viscosity

A

BLOOD VISCOSITY

29
Q

What investigation must be done in PRV

A

JAK2 mutation status

30
Q

what is a JAK2 mutation

A

JAK2 is a kinase

mutations present in over 95% of PRV

31
Q

effects of JAK2 mutation

A

loss of auto-inhibition

activation of erythropoiesis in the absence of ligand

32
Q

treatment of PRV

A

venesection to get haematocrit <0.45
aspirin
cytotoxic oral chemotherapy

33
Q

example of cytotoxic oral chemo drug used in PRV

A

hydroxycarbamide

34
Q

what is essential thrombocytosis

A

uncontrolled production of abnormal platelets

  • thrombosis
  • high levels can cause bleeding — acquired von willebrand
35
Q

clinical features of essential thrombocytosis

A

bleeding

vaso-occlusive complications

36
Q

what should be excluded in essential thrombocytosis

A

exclude reactive thrombocytosis
- blood loss, inflammation, malignancy, iron deficiency
(this is more common)

37
Q

what genetic mutations are tested in essential thrombocytosis

A

JAK2- +ve in 50%
CALR mutation
MPL mutation

38
Q

treatment of essential thrombocytosis

A

anti-platelet – aspirin

cytoreductive therapy to control proliferation

  • hydroxycarbamide
  • interferon alpha
39
Q

what is myelofibrosis

A

clonal proliferation of abnormal megakaryocytes that release fibroblast stimulating factors e.g. PDGF (platelet derived growth factor)
this leads to myelofibrosis

40
Q

what fibroblast stimulating factor is high in myelofibrosis

A

Platelet derived growth factor- PDGF

41
Q

features of myelofibrosis

A

marrow failure – anaemia, infections, bleeding
extra medullary haemopoiesis – massive splenomegaly
hyper metabolism – weight loss, fever, sweats, fatigue

42
Q

film appearances of idiopathic myelofibrosis

A

leukoerythroblastic

- precursors of white cells and red cells seen on blood film

43
Q

characteristic shape of red blood cells in idiopathic myelofibrosis

A

teardrop shaped RBCs in peripheral blood

44
Q

symptoms of myelofibrosis

A

Marrow failures

  • anaemia
  • bleeding
  • infection

splenomegaly

  • LUQ pain
  • portal hypertension

Hypermetabolism

45
Q

investigations + findings of myelofibrosis

A

blood film - tear drop RBCs

dry aspirate- due to the scarring/fibrosis in the bone marrow

fibrosis on trephine biopsy

JAK2 or CALR mutation

46
Q

causes of a leucoerythroblastic film

A

reactive (sepsis)
marrow infiltration
myelofibrosis

47
Q

treatment of myelofibrosis

A

supportive

  • blood transfusion
  • platelets
  • antibiotics

allogenic stem cell transplantation

JAK2 inhibitors

48
Q

what common drug can cause increased neutrophils

A

steroids - disrupts margination and causes redistribution

49
Q

what physiological state can increase neutrophils

A

pregnancy

50
Q

reactive causes of thrombocytosis

A 
infection
chronic inflammation e.g. RA/IBD
anaemia 
hyposplenism 
haemorrhage
51
Q

why is gout seen in myeloproliferative disorders

A

increased cell turnover + increased uric acid production

52
Q

symptoms due to increased blood viscosity in polycythaemia vera

A

headache
tinnitus
visual disturbance

Haematology Year 3 (21 decks)

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