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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?

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
What is the mainstay treatment for PRV?
- Venesect to keep the haematocrit <45%.0.45 - Aspirin reduces stroke risk - Hydroxycarbamide - reduces risk of thrombosis in >60 y/o
26
What is the life expectancy for a patient with polycythaemia rubra vera?
Should be normal
27
What is essential thrombocythaemia?
A clonal proliferation of megakaryocytes leading to persistently high levels of abnormally functioning platelets.
28
What are some of the consequences of essential thrombocythaemia?
- Arterial or Venous Thrombosis | - Bleeding (due to acquired von Willebrand)
29
List some of the symptoms of essential thrombocythaemia?
Headache Atypical chest pain Light headedness Erythromelalgia
30
What are some causes of thrombocytosis that need to be ruled out when diagnosing essential thrombocythaemia?
``` must rule out REACTIVE THROMBOCYTOSIS including: Bleeding Infection Chronic inflammation Malignancy Trauma post surgery Iron deficiency ```
31
How is essential thrombocythaemia managed?
Aspirin | Cytoreductive therapy to reduce proliferation e.g. hydroxycarbamide, anagrelide, interferon alpha
32
In polycythaemia rubra vera what needs to be excluded ?
Secondary polycythaemia or Pseudopolycythaemia
33
What causes pseupolycythaemia?
Dehydration Diuretics Obesity
34
What causes secondary polycythaemia?
Chronic hypoxia Smoking Erythropoeitin secreting tumor
35
What is myelofibrosis?
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
What findings are seen in idiopathic myelofibrosis?
``` Marrow Failure Bone marrow fibrosis Extramedullary haematopoiesis Tear drop shaped RBCs JAK2 mutation Leukoerythroblastic film (VI!) ```
37
List some of the clinical features of myelofibrosis?
``` Anemia Bleeding Infection Splenomegaly LUQ pain Hypercatabolism Portal hypertension ```
38
List the lab diagnosis findings for myelofibrosis:
``` Tear drop blood film Dry aspirate Fibrosis on trephine biopsy JAK2 mutation OR CALR mutation ```
39
What causes a leukoerythroblastic film?
Reactive (sepsis) Marrow infiltration Myelofibrosis
40
What treatments are there for myelofibrosis?
Mainly supportive e.g. transfusions Allogeneic stem cell transplant Splenectomy (controversial) JAK2 inhibitors
41
What is the median survival for myelofibrosis?
4-5 years
42
What age range is myelofibrosis more common in?
>50 y/o
43
What are the treatment options for an asymptomatic patient with myelofibrosis?
Folic acid and pyridoxine supplements Allopurinol Peginterferon alpha
44
A myelofibrosis patient <50 y/o who is symptomatic can receive what type of treatment (if suitable)?
Myeloablative stem cell transplant
45
A myelofibrosis patient >50y/o who is symptomatic can receive what type of treatment (if suitable)?
Non-myeloablative stem cell transplant
46
List some of the complications associated with primary myelofibrosis:
``` Myeloid metaplasia Anaemia infections Portal hypertension Haemorrhage Acute leukaemia Hyperuricaemia related gout / renal stones ```

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