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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
causes of secondary polycythaemia
hypoxia - copd - smoking - altitude Inappropriate EPO production erythropoietin secreting tumour - liver, renal, cerebellar
26
pseudopolycythaemia
``` decreased plasma volume therefore relative increase in concentration caused by: dehydration diuretic therapy obesity ```
27
specific features of PRV
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
what is raised in PRV: blood viscosity or plasma viscosity
BLOOD VISCOSITY
29
What investigation must be done in PRV
JAK2 mutation status
30
what is a JAK2 mutation
JAK2 is a kinase | mutations present in over 95% of PRV
31
effects of JAK2 mutation
loss of auto-inhibition | activation of erythropoiesis in the absence of ligand
32
treatment of PRV
venesection to get haematocrit <0.45 aspirin cytotoxic oral chemotherapy
33
example of cytotoxic oral chemo drug used in PRV
hydroxycarbamide
34
what is essential thrombocytosis
uncontrolled production of abnormal platelets - thrombosis - high levels can cause bleeding --- acquired von willebrand
35
clinical features of essential thrombocytosis
bleeding | vaso-occlusive complications
36
what should be excluded in essential thrombocytosis
exclude reactive thrombocytosis - blood loss, inflammation, malignancy, iron deficiency (this is more common)
37
what genetic mutations are tested in essential thrombocytosis
JAK2- +ve in 50% CALR mutation MPL mutation
38
treatment of essential thrombocytosis
anti-platelet -- aspirin cytoreductive therapy to control proliferation - hydroxycarbamide - interferon alpha
39
what is myelofibrosis
clonal proliferation of abnormal megakaryocytes that release fibroblast stimulating factors e.g. PDGF (platelet derived growth factor) this leads to myelofibrosis
40
what fibroblast stimulating factor is high in myelofibrosis
Platelet derived growth factor- PDGF
41
features of myelofibrosis
marrow failure -- anaemia, infections, bleeding extra medullary haemopoiesis -- massive splenomegaly hyper metabolism -- weight loss, fever, sweats, fatigue
42
film appearances of idiopathic myelofibrosis
leukoerythroblastic | - precursors of white cells and red cells seen on blood film
43
characteristic shape of red blood cells in idiopathic myelofibrosis
teardrop shaped RBCs in peripheral blood
44
symptoms of myelofibrosis
Marrow failures - anaemia - bleeding - infection splenomegaly - LUQ pain - portal hypertension Hypermetabolism
45
investigations + findings of myelofibrosis
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
causes of a leucoerythroblastic film
reactive (sepsis) marrow infiltration myelofibrosis
47
treatment of myelofibrosis
supportive - blood transfusion - platelets - antibiotics allogenic stem cell transplantation JAK2 inhibitors
48
what common drug can cause increased neutrophils
steroids - disrupts margination and causes redistribution
49
what physiological state can increase neutrophils
pregnancy
50
reactive causes of thrombocytosis
``` infection chronic inflammation e.g. RA/IBD anaemia hyposplenism haemorrhage ```
51
why is gout seen in myeloproliferative disorders
increased cell turnover + increased uric acid production
52
symptoms due to increased blood viscosity in polycythaemia vera
headache tinnitus visual disturbance

Haematology Year 3 (21 decks)

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