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Haematology SB > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

what is meant by myeloproliferative

A

abnormal proliferation of myelo cells in the marrow

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2
Q

what is definition of CMD

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 CMD differ on blood film

A

acute leukaemia - lots of primitive cells

MPD = numbers are increasing but do have normal maturations

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4
Q

how is CMD subdivided

A

BCR-ABL 1 negative

  • idiopathic myelofibrosis
  • essential thrombocythaemia (ET)
  • Polycythaemia Rubra Vera (PRV)

BCR-ABL 1 positive
- CML [philadelphia chromsome]

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5
Q

what is the basic problem in ET

A

over production of platelets

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6
Q

what is the basic problem in PRV

A

over production of red cells

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7
Q

when should MPD be consider - in terms of blood results

A 
High Granulocyte count
				±
High Red cell count / haemoglobin
				±
High Platelet count
				±
Eosinophilia/basophilia

Splenomegaly

Thrombosis in an unusual place

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8
Q

what is CML

A

Proliferation of myeloid cells

  • Granulocytes & their precursors
  • Other lineages (platelets)

Fatal without stem cell/bone marrow transplant in the chronic phase

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9
Q

what is the pathology of CML

A

chronic phase with intact maturation 3-5 years, followed by ‘blast crisis’ reminiscent of acute leukaemia with maturation defect

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10
Q

what are Sx of CML

A 
asymptomatic 
splenomegaly
hypermetabolic Sx
gout 
Priapism [persistent and painful erection]
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11
Q

what might people complain of if they have splenomegaly

A

people feel hungry, and then they start eating and then they feel full quickly

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12
Q

what are lab finding of CML

A

normal/low Hb

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

thrombocytosis (too many platelets)

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13
Q

what is the hallmark of CML

A

philadelphia chromosome

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14
Q

what is philadelphia chromosome

A

part of one chromosome moving to another

Chr 22 gives some information to Chr 9

Makes the BCR-ABL 1 gene

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15
Q

what does the BCR-ABL 1 gene cause

A

tyrosine kinase which causes abnormal phosphorylation (signalling) leading to the haematological changes in CML

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16
Q

what is the Tx for CML

A

Imatinib

17
Q

what are Sx are common to all BCR-ABL1 Negative MPD

A

Asymptomatic

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

Splenomegaly Sx

Marrow failure

Thrombosis
- arterial or venous including TIA, MI, claudication, erythromelalgia [thrombosis in hands and feet]

18
Q

what are lab findings of PRV

A

High Hb accompanied by erythrocytosis (true increase in red cell mass)

Can also have an excessive production of other lineages

19
Q

what must PRV be distinguished from

A

secondary polycythaemia - chronic hypoxia, smoking, bad asthma, erythropoietin-secreting tumour

pseudopolycythaemia -dehydration, diuretic therapy, obesity

20
Q

what are features specific to PRV

A
  • features common to MPD
  • headache, fatigue
  • itch (aquagenic puritis - patient has a shower and then have an uncontrollable itch for an hour or so)
21
Q

what Ix must be done to PRV

A

JAK2 mutation status

22
Q

what is JAK2 mutation in PRV

A

Mutation (substitution) results in loss of auto-inhibition

Activation of erythropoiesis in the absence of ligand

23
Q

what is Tx for PRV

A

vensect to haematocrit < 4.45

Aspirin

Cytotoxic oral chemotherapy (eg Hydroxycarbamide)

24
Q

what is ET

A

Uncontrolled production of abnormal platelets

  • thrombosis
  • at high levels can also cause bleeding due to acquired von Willebrand disease
25
Q

what are Sx of ET

A
  • Sx common to MPD
  • vaso-occlusive complications
  • Bleeding (unpredictable esp at surgery)
26
Q

what should be excluded in ET

A

Exclude reactive thrombocytosis
- blood loss, inflammation, malignancy, iron deficiency

Exclude CML

27
Q

what genetics mutations are tested in ET

A

JAK2 mutation - +ve in 50%

CALR (Calreticulin) - those w/out JAK2

MPL mutation

28
Q

what is type last Ix in ET

A

Bone marrow aspirate

29
Q

what is Tx of ET

A

Anti-platelet - Aspirin

Cytoreductive therapy to control proliferation
- hydroxycarbamide, anagrelide, interferon alpha

30
Q

what is myelofibrosis

A

rare bone marrow cancer

proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis, or the replacement of the marrow with scar tissue

31
Q

what are the two mechanisms of myelofibrosis

A

Idiopathic

Post-polycythaemia or post-essential thrombocythaemia

32
Q

what are features of IDIOPATHIC myelofibrosis

A

Marrow failure
Bone marrow fibrosis
Extramedullary haetopoiesis

33
Q

what are the film appearances of idiopathic myelofibrosis

A

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

34
Q

what are characteristic about the shape of red blood cells in idiopathic myelofibrosis

A

Teardropped shaped RBCs

35
Q

what are Sx of MF

A

Marrow failure
- anaemia, bleeding, infection

Splenomegaly
- LUQ abdo pain, portal hypertension

Hypercatabolism

36
Q

what are IX of MF

A

blood film - tear-drop shaped RBC and leucoerythroblastic

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

fibrosis on trephine biopsy

JAK2 or CALR mutation in a proportion

37
Q

when are Leucoerythroblastic film seen

A

Reactive (sepsis)
Marrow infiltration
MF

38
Q

what is the Tx of MF

A

Supportive care - blood transfusion, platelets, antibiotics

Allogeneic stem cell transplantation in a select few

Splenectomy

JAK2 inhibitors e.g. Ruxolitinib therapy

Haematology SB (19 decks)

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