Polycythaemia Vera Flashcards

1
Q

What is polycythaemia vera?

A

It is defined as a myeloproliferative disorder in which there is excess production of erythrocytes

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2
Q

What is the pathophysiological consequence of polycythaemia vera?

A

It results in raised haemoglobin concentration, increased red cell volume and haematocrit

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3
Q

What is the pathophysiological cause of polycythaemia vera?

A

JAK2 Mutation

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4
Q

What two risk factors are associated with polycythaemia vera?

A

Older Age, 60 -70 Years Old

Budd-Chiari Syndrome

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5
Q

What are the ten clinical features of polycythaemia vera?

A

Fatigue

Headaches

Ruddy Complexion

Erythromelalgia

Pruritis

Tinnitus

Abdominal Mass

Splenomegaly

Haemorrhage

Hypertension

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6
Q

What is erythomelalgia?

A

It is defined as burning pain, warmth and redness in the hands and feet

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7
Q

Describe the pruritus associated with polycythaemia vera

A

It tends to occur or worsen following hot showers/baths

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8
Q

What four investigations are used to diagnose polycythaemia vera?

A

Blood Tests

Arterial Blood Gases (ABG)

Abdominal Ultrasound

Bone Marrow Biopsy

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9
Q

What ten blood test results indicate polycythaemia vera?

A

Increased Haemoglobin Levels

Increased Haematocrit

Increased Red Cell Mass > 25%

Increased Platelet Levels

Increased Neutrophil Levels

Increased Basophil Levels

Decreased ESR Levels

Increased ALP Levels

Normal/Decreased Ferritin Levels

Positive JAK2 Analysis

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10
Q

What haemoglobin level result indicated polycthaemia - in males/females?

A

Males = > 185g/L

Females = > 165g/L

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11
Q

What haematocrit result indicates polycythaemia vera - in males/females?

A

Males = > 0.52

Females = > 0.48

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12
Q

What is secondary polycythaemia?

A

It is defined as increased erythrocytes due to physiological response to chronic hypoxia, local renal hypoxia or excess erythropoietin production

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13
Q

What investigation is used to differentiate between polycythaemia vera and secondary polycythaemia? Explain

A

Total red cell mass

In polycythaemia vera, the total red cell mass is > 35ml/kg in males and > 32ml/kg in females

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14
Q

What two ABG features indicate polycythaemia vera?

A

Decreased Oxygen Levels

Increased Carboxyhaemoglobin Levels

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15
Q

How are abdominal ultrasounds used to investigate polycythaemia vera?

A

They are used to identify splenomegaly and exclude secondary causes of polycythaemia, such as renal and hepatic pathology

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16
Q

How are bone marrow biopsies used to investigate polycythaemia vera?

A

They are used to distinguish polycythaemia vera from secondary polycythaemia

17
Q

What are the five features of polycythaemia vera on bone marrow biopsies?

A

Hypercellularity

Erythropoiesis

Granulopoiesis

Megakaryopoiesis

Variable Megakaryote Size

18
Q

What are the two diagnostic criteria of JAK-2 positive polycythaemia vera?

A

Increased Haematocrit (0.52 males, 0.48 females) OR Increased Red Cell Mass

AND

JAK2 Mutation

19
Q

What are the nine diagnostic criteria of JAK-2 negative polycythaemia vera?

A

The individual must meet A1 - A4 plus two other criteria…

A1 = Increased Haematocrit (0.60 males, 0.56 females) OR Increased Red Cell Mass

A2 = Negative JAK2 Mutation

A3 = No Secondary Erythrocytosis Cause

A4 = Bone Marrow Histology Positive

A5 = Palpable Splenomegaly

A6 = Acquired Genetic Abnormality In Haematopoietic Cells

B1 = Thrombocytosis > 550 x 109/L

B2 = Neutrophil Leucocytosis > 10 x109/L Non-Smokers, > 12.5 x 109/L Smokers

B3 = Radiological Splenomegaly

20
Q

What is the conservative management option of polycythaemia vera?

A

It involves cardiovascular risk factor optimisation, due to the increased risk of arterial/venous thrombosis

21
Q

What is the pharmacological management option of polycythaemia vera?

22
Q

When is aspirin used to manage polycythaemia vera?

A

It is used to manage all polycythaemia vera patients

23
Q

How is aspirin used to manage polycythaemia vera?

A

In order to reduces the risk of thrombotic events

24
Q

When is venesection used to manage polycythaemia vera?

A

It is the first line management option of polycythaemia vera

25
What is venesection?
It involves withdrawing around 200-500mls of blood at intervals suitable to the patient
26
What is the aim of vensection in polcythaemia vera?
Haematocrit levels < 0.45
27
When is cytoreductive therapy used to manage polycythaemia vera?
It is the second line management option of polycythaemia vera, which is indicated in those who meet the following criteria... * Age > 60 Years Old * Progressive Leucocytosis > 15 * Progressive Thrombocytosis > 1500 * Progressive Splenomegaly * Poor Venesection Tolerance * Thrombosis History
28
How is cytoreductive therapy used to manage polycythaemia vera?
It is used to reduce the white cell and platelet count
29
What is the first line cytoreductive therapy used to manage polycythaemia vera?
Hydroxycarbamide
30
What is the second line cytoreductive therapy used to manage polycythaemia vera?
Interferon-alfa
31
What is the first line cytoreductive therapy agent in women of childbearing age? Why?
Interferon-alfa This is due to its lack of teratogenic effects, compared to hydroxycarbamide
32
What are the six complications associated with polycythaemia vera?
Ischaemic Stroke Myocardial Infarction Pulmonary Embolism Myelofibrosis Acute Myeloid Leukaemia Budd-Chiari Syndrome