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Haematology > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

What is meant by a myeloproliferative disorder?

A

Clonal haemopoietic stem cell disorders with an increased production of on or more types of haemopoietic cells. Maturation is relatively preserved, unlike acute leukemia

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2
Q

What are the two subtypes of myeloproliferative disorders?

A
  1. BCR - ABL1 Positive (Chronic Myeloid Leukemia)

2. BCR ABL1 Negative

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3
Q

What chromosome is mutated in CML?

A

Philadelphia chromosome

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4
Q

What is the problem in the philadelphia chromosome?

A

Balanced translocation from chromosome 9 to chromosome 22

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5
Q

What are the three stages on CML?

A

Chronic
Accelerated
Blast crisis

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6
Q

What clinical features are you looking for in CML?

A

Splenomegaly
Hypermetabolic syndrome
Gout

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7
Q

What will the blood count look like in CML?

A

Normal/low Hb

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

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8
Q

What does the philidelphia chromosome cause?

A

A new gene - BCR - ABL 1

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9
Q

What is the gene product of the philidelphia chromosome and what does it do?

A

Tyrosine kinase which causes abnormal signalling and the haematological changes seen in CML

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10
Q

What is the treatment for CML and how does it work?

A

Tyrosine kinase inhibitor (Imatinib)

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11
Q

What are the three BRC - AB41 negative myeloproliferative disorders?

A

Polycythaemia rubra vera
Essential thrombocythaemia
Idiopathic myelofibrosis

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12
Q

What are the causes of secondary polycythaemia?

A

Chronic hypoxia (eg in COPD)
Smoking
EPO secreting tumout

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13
Q

What is a cause of pseudopolycythaemia?

A

Dehydration (Decreased plasma volume with result in disproportionate red cell numbers)

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14
Q

A patient presents with headaches, fatigue and says they get very itchy after every bath or shower.

A

Polycythaemia Rubra Vera

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15
Q

What is the most important test to do to confirm a diagnosis of Polycythemia Rubra Vera?

A

JAK2 mutation status

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16
Q

What is the genetic mutation see in almost all patients with Polycythemia Rubra Vera?

A

JAK2

17
Q

What % of patients with Polycythemia Rubra Vera have the JAK2 gene?

A

95%

18
Q

What is JAK2 and what does it do?

A

A kinase that results causes a loss of auto inhibitions

19
Q

List all the main investigations you would do for Polycythemia Rubra Vera

A 
FBC 
Blood film
JAK2 status
CXR
02 sats
20
Q

What is the treatment of Polycythemia Rubra Vera?

A

Venesection
Aspirin
Cytotoxic oral chemotherapy (Hydroxycarbamide)

21
Q

What is essential thrombocythaemia?

A

Uncontrolled production of abnormal platelets

22
Q

What symptoms do patients get in essential thrombocythaemia?

A 
Thrombosis
Bleeding (due to acquired von willebrend disease) 
Microvascular Occlusion 
Gout
Fatigue
23
Q

How do you diagnose essential thrombocythaemia?

A

Exclude reactive cause (blood loss, inflammation, malignancy, iron deficiency)
JAK2 mutation

24
Q

What is the treatment for essential thrombocythaemia?

A

Treatment dependent on age (younger patients most likely don’t need any treatment) but older patients need anti thrombotic agents such as aspirin.
Cytoreductive therapy to control proliferation (hydroxycarbamide)

25
Q

What is myelofibrosis?

A

A condition in which an abnormal clone of haemopoietic stem cells in the bone marrow and other sites results in fibrosis, or the replacement of bone marrow with scar tissue

26
Q

What is leukoerythroblastic change?

A

Nucleated red blood cells and primitive white cells seen in the peripheral blood

27
Q

How does myelofibrosis present?

A

Marrow failure (bleeding, infection)
Splenomegaly
Hypercatabolism

28
Q

In which conditon do you see teardrop shaped red blood cells?

A

myelofibrosis

29
Q

What important changes to the blood film do you see in myelofibrosis?

A

Leucoerythroblastic change

Tear drop red blood cells

30
Q

What important changes do you see on a trephine biopsy of myelofibrosis?

A

Fibrosis

31
Q

What is the treatment for myelofibrosis?

A

Supportive (transfusion, platelets)
Allogenic stem cell transplant
JAK2 inhibitors

Haematology (21 decks)

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