Myeloproliferative disorders Flashcards

1
Q

Myeloproliferative - definition

A

Proliferation of myeloid cells within the bone marrow

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2
Q

Myeloid cells within the bone marrow - categories

A

Granulocytes
Red cells
Platelets

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3
Q

Histology

A

Mixture of different cell types

Hypercellular

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4
Q

What are the 2 subtypes

A

BCR-ABL1 negative

BCR-ABL1 positive

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5
Q

Which 3 conditions are BCR-ABL1 negative?

A

Polycythaemia Rubra Vera
Essential thyombocythaemia
Idiopathic myelofibrosis

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6
Q

What condition is BCR-ABL1 positive?

A

Chronic myeloid leukaemia

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7
Q

When should you consider MPD as a diagnosis?

A

When there is NO reactive (secondary) explanation

High granulocyte count +/- 
High RBC count / Hb +/-
High platelet count +/- 
Eosinophilia/basophilia +/-
Splenomegaly
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8
Q

MPD is always a primary problem in the bone marrow itself. True or false?

A

True

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9
Q

Eosinophilia/basophilia is more likely to indicate primary or secondary problem

A

Primary (MPD)

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10
Q

If patient has bloods taken and the results are significantly abnormal, what is the course of action?

A

Re-check the blood count to make sure there haven’t been any errors

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11
Q

Clinical features

A

Asymptomatic

Increased cell turnover
- gout, fatigue, weight loss, sweats

Symptoms/signs due to splenomegaly

erythromelalgia

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12
Q

Polycythaemia rubra vera - blood count features

A

High Hb / HCT
Erythrocytosis (too many red cells)
Low EPO levels
May have excessive production of other lineages

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13
Q

Secondary polycythaemia - features

A

Chronic hypoxia
Smoking
EPO-secreting tumour

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14
Q

Pseudopolycythaemia

A

Normal amount of RBC but on blood count it appears to be high as it is relative to a reduction in the plasma volume

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15
Q

Polycythaemia rubra vera - clinical features

A
Common to MPD 
Headache 
Fatigue 
Itch (aquagenic puritis) 
- worse on exposure to warm water
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16
Q

Patient with an itch that is worse on exposure to warm water has which condition?

A

Polycythaemia rubra vera

17
Q

Polycythaemia rubra vera - genetics

A

JAK2 mutation

- present in over 95% of patients

18
Q

Polycythaemia rubra vera - mutational analysis forms part of initial screening. True or false?

A

True

19
Q

Polycythaemia rubra vera - management

A

Venesect to haematocrit <0.45 (ie - take pints of blood off)

Aspirin (too many red cells means they are more likely to thrombose)

Cytotoxic oral chemo

20
Q

Essential thrombocythaemia - definition

A

Too many platelets

  • also, platelets are larger than normal
  • platelets are ABNORMAL
21
Q

Essential thrombocythaemia - clinical features

A

Common to MPD
Bleeding
Digital ischaemia

22
Q

Common reactive reason for high platelet count

A

Churning out platelets to combat the haemostatic challenge they have had with past

23
Q

Essential thrombocythaemia - investigations

A

JAK2 mutations (in 50%)

24
Q

JAK2 mutations are specific to PRV. True or false?

A

False

25
Q

Essential thrombocythaemia - if the patient doesn’t have a JAK2 mutation, then what mutation may they have

A

CALCR mutation

26
Q

Essential thrombocythaemia - management

A

Anti-platelets eg aspirin

Cytoreductive therapy to control proliferation

  • hydroxycarbomide
  • anagrelide
  • interferon alpha
27
Q

myelofibrosis - types

A

Idiopathic

Post-polycythaemic or

28
Q

Myelofibrosis - definition

A

Marrow failure
Scarring in the bone marrow
Extramedullary haematopoiesis

29
Q

Myelofibrosis - blood film appearance

A

Leukoerythroblastic

Tear drop shaped RBCs in peripheral blood

30
Q

Myelofibrosis - clinical features

A

Marrow failure

  • anaemia
  • bleeding
  • infection

Splenomegaly

  • LUQ pain
  • complications

Hypercatabolism

31
Q

Myelofibrosis - what can you do instead of bone marrow aspiration?

A

Trephine biopsy

32
Q

Leucoerythroblastic film - causes

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

33
Q

What is a leucoerythroblastic film composed of?

A

Myelocytes

Erythroblast (nucleated RBC)

34
Q

Myelofibrosis - management

A

Supportive

  • blood transfusion
  • platelets
  • antibiotics

Allogenic stem cell transplantation in select few

Splenectomy

JAK 2 inhibitors

35
Q

Reactive causes of high counts are more common than MPD. True or false?

A

True