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Haematology > Chronic Myeloproliferative Disorders (MPD) > Flashcards

Chronic Myeloproliferative Disorders (MPD) Flashcards

1
Q

Define myeloproliferative disorders:

A

Group of diseases of clonal haemopoietic stem cells where there is increased proliferation with fairly normal maturation.

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2
Q

List the main MPDs:

A

Chronic myeloid leukemia
Polycythaemia Vera
Myelofibrosis
Essential thrombocythaemia

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3
Q

What sub-type does chronic myeloid leukemia fall under?

A

BCR-ABL1 Positive

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4
Q

What sub-type does idiopathic myelofibrosis fall under?

What other disorders also fall under this sub-type?

A

BCR-ABL1 Negative

  • Polycythaemia Rubra Vera
  • Essential thrombocythaemia
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5
Q

What is polycythaemia rubra vera?

A

Over production of red blood cells

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6
Q

What is chronic myeloid leukemia?

A

Over production of granulocytes

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7
Q

What features should make you consider a MPD?

A

All explainable –>

  • High granulocyte count
  • High RBC count
  • High platelet count
  • Eosinophilia / basophilia
  • Splenomegaly
  • Thrombosis in unusual places
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8
Q

In chronic myeloid leukemia, what is a ‘blast crisis’?

A

This occurs a few years after the initial, chronic phase of the ML. It is an accelerated phase where the blood counts become difficult to control due to a maturation defect.
It is defined by an increasing number of blasts in the bone marrow

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9
Q

What genetic defect is chronic myeloid leukemia associated with?

A

‘Philadelphia chromosome ‘
Reciprocal translocation between C22 and C9 which leads to the development of tyrosine kinase
(BRC-ABL1)

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10
Q

List some of the clinical features of chronic myeloid leukemia

A 
Can be asymptomatic 
Fatigue 
Weight loss
night sweats
Anorexia 
Pallor
Splenomegaly
gout
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11
Q

List some symptoms associated with hyperleucocytosis in chronic myeloid leukemia:

A

Priapism
tinnitus
Stupor

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12
Q

What lab features would be seen in a patient with chronic myeloid leukemia?

A 
Normal / slight decreased Hb
Leucocytosis with neutrophils and myeloid precursors
Eosiniphilia
Basophilia
Thrombocytosis
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13
Q

What is the mechanism of action of imatinib

A

Tyrosine Kinase inhibitor

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14
Q

What is the median life expectancy of chronic myeloid leukemia?

A

5-6 years

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15
Q

What is the main treatments for CML?

A

1st line:
Imatinib
2nd Line:
Dasatinib, nilotinib, bosutinib

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16
Q

What are some of the side effects of imatinib.

A 
Nausea
Cramps
Oedema
Rash
Headache
Arthralgia
May cause myelosuppression
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17
Q

What is polycythaemia rubra vera? (PRV)

A

A chronic clonal disorder where excessive proliferation leads to increased RBCs.
The the haemoglobin : haematocrit ratio is high and there is erythrocytosis
(they can also have excess of other lineages)

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18
Q

Chronic myeloid leukemia is commoner in young/older patients?

A

Older

extremely rare in children

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19
Q

What are the main presenting symptoms of polycythaemia rubra vera?

A 
Headache
Fatigue
Dizziness
Pruritis (esp. after hot bath)
Plethora
20
Q

What are the main clinical signs of polycythaemia rubra vera?

A

Splenomegaly
Hepatomegaly
Increased skin temp
burning sensation and redness in the peripheries

21
Q

A mutation in what gene has been discovered in roughly 95% of patients with PRV?

A

JAK 2

22
Q

What is JAK 2?

A

A kinase whose mutations result in the loss of auto-inhibition

23
Q

What are the typical lab findings of a patient with polycythaemia rubra vera?

A

Raised Hb
Raised PCV
Can have increased WCC
Increased haematocrit

24
Q

What age range is typically affected by polycythaemia rubra vera?

A

Over 60

<40 is very rare

25
Q

What is the mainstay treatment for PRV?

A
  • Venesect to keep the haematocrit <45%.0.45
  • Aspirin reduces stroke risk
  • Hydroxycarbamide - reduces risk of thrombosis in >60 y/o
26
Q

What is the life expectancy for a patient with polycythaemia rubra vera?

A

Should be normal

27
Q

What is essential thrombocythaemia?

A

A clonal proliferation of megakaryocytes leading to persistently high levels of abnormally functioning platelets.

28
Q

What are some of the consequences of essential thrombocythaemia?

A
  • Arterial or Venous Thrombosis

- Bleeding (due to acquired von Willebrand)

29
Q

List some of the symptoms of essential thrombocythaemia?

A

Headache
Atypical chest pain
Light headedness
Erythromelalgia

30
Q

What are some causes of thrombocytosis that need to be ruled out when diagnosing essential thrombocythaemia?

A 
must rule out REACTIVE THROMBOCYTOSIS
including:
Bleeding
Infection
Chronic inflammation
Malignancy
Trauma
post surgery
Iron deficiency
31
Q

How is essential thrombocythaemia managed?

A

Aspirin

Cytoreductive therapy to reduce proliferation e.g. hydroxycarbamide, anagrelide, interferon alpha

32
Q

In polycythaemia rubra vera what needs to be excluded ?

A

Secondary polycythaemia
or
Pseudopolycythaemia

33
Q

What causes pseupolycythaemia?

A

Dehydration
Diuretics
Obesity

34
Q

What causes secondary polycythaemia?

A

Chronic hypoxia
Smoking
Erythropoeitin secreting tumor

35
Q

What is myelofibrosis?

A

A clonal disorder which leads to hyperplasia of megakaryocytes (fibroblasts) that produce platelet growth factor, leading to fibrosis of the bone marrow and myeloid dysplasia.

36
Q

What findings are seen in idiopathic myelofibrosis?

A 
Marrow Failure
Bone marrow fibrosis
Extramedullary haematopoiesis
Tear drop shaped RBCs
JAK2 mutation
Leukoerythroblastic film (VI!)
37
Q

List some of the clinical features of myelofibrosis?

A 
Anemia
Bleeding
Infection
Splenomegaly
LUQ pain
Hypercatabolism
Portal hypertension
38
Q

List the lab diagnosis findings for myelofibrosis:

A 
Tear drop blood film
Dry aspirate
Fibrosis on trephine biopsy
JAK2 mutation 
OR
CALR mutation
39
Q

What causes a leukoerythroblastic film?

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

40
Q

What treatments are there for myelofibrosis?

A

Mainly supportive e.g. transfusions
Allogeneic stem cell transplant
Splenectomy (controversial)
JAK2 inhibitors

41
Q

What is the median survival for myelofibrosis?

A

4-5 years

42
Q

What age range is myelofibrosis more common in?

A

> 50 y/o

43
Q

What are the treatment options for an asymptomatic patient with myelofibrosis?

A

Folic acid and pyridoxine supplements
Allopurinol
Peginterferon alpha

44
Q

A myelofibrosis patient <50 y/o who is symptomatic can receive what type of treatment (if suitable)?

A

Myeloablative stem cell transplant

45
Q

A myelofibrosis patient >50y/o who is symptomatic can receive what type of treatment (if suitable)?

A

Non-myeloablative stem cell transplant

46
Q

List some of the complications associated with primary myelofibrosis:

A 
Myeloid metaplasia
Anaemia
infections
Portal hypertension
Haemorrhage
Acute leukaemia 
Hyperuricaemia related gout / renal stones

Haematology (13 decks)

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