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Haematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What are myeloproliferative disorders?

A

Conditions which occur due to uncontrolled proliferation of a single type of stem cell.

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2
Q

What are myeloproliferative disorders considered to be?

A

A type of bone marrow cancer

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3
Q

What are the three main myeloproliferative disorders?

A
  • Primary myelofibrosis
  • Polycythaemia vera
  • Essential thrombocythaemia
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4
Q

What is primary myelofibrosis the result of?

A

The result of proliferation of the hematopoietic stem cells.

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5
Q

What is polycythaemia vera the result of?

A

The result of proliferation of the erythroid cell line.

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6
Q

What is essential thrombocythemia the result of?

A

The result of proliferation of the megakaryocytic cell line.

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7
Q

What do Myeloproliferative disorders have the potential to do?

A

Progress and transform into acute myeloid leukaemia.

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8
Q

What gene mutations are these conditions associated with?

A
  • JAK2
  • MPL
  • CALR
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9
Q

What gene mutation do the majority of people with polycythaemia vera have?

A

JAK2 mutation

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10
Q

What is myelofibrosis though to be caused by?

A

Hyperplasia of abnormal megakaryocytes

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11
Q

What can myelofibrosis be the result of?

A
  • Primary myelofibrosis
  • Polycythaemia vera
  • Essential thrombocythemia.
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12
Q

What does the proliferation of the cell line lead to in myelofibrosis?

A

It leads to fibrosis of the bone marrow.

The bone marrow is replaced by scar tissue.

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13
Q

What is fibrosis of the bone marrow the result of?

A

Cytokines that are released from the proliferating cells.

Especially cytokine- fibroblast growth factor.

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14
Q

What does fibrosis of the bone marrow affect?

A

The production of blood cells and can lead to anaemia and low white blood cells (leukopenia).

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15
Q

Where does the production of blood cells start to occur when there is fibrosis of the bone marrow?

A

Liver and Spleen

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16
Q

What is the name of the production of red blood cells in the spleen and liver?

A

Extramedullary haematopoiesis

17
Q

What can extramedullary haematopoiesis result in?

A

hepatomegaly and splenomegaly

18
Q

How do myeloproliferative disorders present?

A
  • Fatigue (most common presenting symptom)
  • Hypermetabolic symptoms: Weight loss, Night sweats, Fever
  • Massive splenomegaly
19
Q

What hypermetabolic symptoms might present with myeloproliferative disorder?

A

Weight loss
Night sweats
Fever

20
Q

What might you find on a blood film with primary myeloproliferative disorder?

A

‘tear-drop’ poikilocytes

21
Q

What might you find with a FBC?

A
  • anaemia

* high WBC and platelet count early in the disease

22
Q

What is the management of mild primary myelofibrosis?

A

Monitored and not actively treated.

23
Q

What is potentially curative for primary myelofibrosis but carries risk?

A

Allogenic stem cell transplant

24
Q

What is the function of chemotherapy in primary myelofibrosis?

A

Helps to control the disease, improve symptoms and slow progression but is not curative alone

25
Q

What are 3 key signs of polycythaemia vera?

A
  • Conjunctival plethora (excessive redness to the conjunctiva in the eyes) (red face)
  • A “ruddy” complexion
  • Splenomegaly
26
Q

What would you find on a full blood count of someone with polycythaemia vera?

A
  • Raised haemoglobin (more than 185g/l in men or 165g/l in women)
  • Primary Thrombocythaemia: Raised platelet count (more than 600 x 109/l)
  • Due to proliferation, raised uric acid
27
Q

What is the first line management of polycythaemia vera?

A

Venesection

28
Q

What is the function of venesection?

A

To keep the haemoglobin in the normal range

29
Q

What can be used to reduce the risk of developing clots?

A

Aspirin

30
Q

What can be used to control the disease of polycythaemia vera?

A

Chemotherapy

31
Q

What is the definitive diagnostic test for polycythaemia vera?

A

Bone marrow biopsy

32
Q

How does a bone marrow biopsy present with polycythaemia vera?

A

Dry- all of the aspiration is usually dry

33
Q

What are the main features of essential thrombocythaemia?

A

platelet count > 600 * 109/l
both thrombosis (venous or arterial) and haemorrhage can be seen
a characteristic symptom is a burning sensation in the hands
a JAK2 mutation is found in around 50% of patients

34
Q

What does megakaryocyte proliferation lead to?

A

Overproduction of platelets

35
Q

What is the medical management of Essential thrombocythaemia?

A

Hydroxycarbamide

35
Q

What is the medical management of essential thrombocythaemia?

A

Hydroxycarbamide

36
Q

What is the function of Hydroxycarbamide?

A

Reduce the platelet count

Haematology (36 decks)

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