Polycythaemia Flashcards

1
Q

Define Polycythaemia?

A

An increase in haemoglobin concentration above the upper limit of normal for a person’s age and sex

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2
Q

How is Polycythaemia classified?

A

Relative Polycythaemia = Normal red cell mass but low plasma volume
Absolute (True) Polycythaemia = increased red cell mass

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3
Q

What is the aetiology of Polycythaemia Rubra Vera?

A

Characterised by clonal proliferation of myeloid cells
They have varied morphologic maturity and haematopoietic efficiency
Mutations in JAK2 tyrosine kinase are involved

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4
Q

What are the two types of Secondary Polycythaemia?

A

Appropriate increase in erythropoietin

Inappropriate inrease in erythropoietin

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5
Q

What is the aetiology of Secondary Polycythaemia where there is an Appropriate increase in erythropoietin?

A

Due to chronic hypoxia (e.g. chronic lung disease, living at high altitude)
This leads to upregulation of erythropoiesis

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6
Q

What are the causes of Secondary Polycythaemia where there is an Inappropriate increase in erythropoietin?

A
Renal (carcinoma, cysts, hydronephrosis)
Hepatocellular carcinoma 
Fibroids 
Cerebellar Haemangioblastoma 
Secondary polycythaemia may be due to erythropoietin abuse by athletes
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7
Q

What is the aetiology of Relative Polycythaemia?

A

Dehydration (e.g. diuretics, burns, enteropathy)

Gaisbock’s Syndrome

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8
Q

What is Gaisbock’s Syndrome?

A

Occurs in young male smokers with hypertension, which results in a decrease in plasma volume and an apparent increase in red cell count

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9
Q

What is the epidemiology of Polycythaemia?

A

Annual UK incidence: 1.5/100,000

Peak age: 45-60 years

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10
Q

What are the presenting symptoms of Polycythaemia?

A
Headaches
Dyspnoea 
Tinnitus 
Blurred Vision 
Pruritis after hot bath 
Night Sweats 
Thrombosis (DVT, Stroke)
Pain from peptic ulcer disease 
Angina 
Gout 
Choreiform movements
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11
Q

What are the signs of Polycythaemia on physical examination?

A

Plethoric complexion (red, ruddy)
Scratch marks from itching
Conjuctival suffusion (redness of the conjuctiva)
Retinal venous engorgement
Hypertension
Splenomegaly (in 75% of cases)
Signs of underlying aetiology in secondary polycythaemia

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12
Q

What investigations do we do for general Polycythaemia?

A

Investigations required for diagnosis

Isotope Dilution Techniques

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13
Q

What investigation do we do for diagnosis of Polycythaemia and what do we see?

A

FBC:

  • High Hb
  • High Haematocrit
  • Low MCV
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14
Q

What are Isotope Dilution Techniques and why do we do it for Polycythaemia?

A

Allows confirmation of plasma volume and red cell mass

Distinguishes between relative and absolute polycythaemia

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15
Q

What investigations do we do for Polycythaemia Rubra Vera?

A
High WCC 
High Platelets 
Low Serum EPO
JAK2 mutation
Bone marrow trephine and biopsy shows erythroid hyperplasia and raised megakaryocytes
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16
Q

What investigations do we do for Secondary Polycythaemia?

A

High Serum EPO
Exclude chronic lung disease/hypoxia
Check for EPO-secreting tumours