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Polycythaemia

Clinical Discipline(s)/Organ System(s)
Haematology, Respiratory Medicine
Progress Test Topic(s)
Blood and lymph, Respiratory
Description
A 54 year old man presents to his GP with headache and lethargy for 2 months. He is overweight with a body mass index (BMI) of 32, and has been smoking a packet of cigarettes each day for the last 20 years. No abnormalities are detected on neurological examination but on examination of the abdomen you can tip the edge of the spleen on deep inspiration. A full blood count shows an elevated haemoglobin of 203 g/L and elevated haematocrit of 0.61. The WBC is normal but platelets are elevated at 603 x 109/L.
Progress Test-Type Questions:   Question 1 | Question 2
Applied Science for Medicine 
   - Development and role of the myeloid cells: red blood cells, platelets, neutrophils, eosinophils and basophils
   - Role of JAK2 and BCR-ABL mutations in the development of myeloproliferative neoplasms
   - Distinction between apparent, primary, and secondary polycythaemia
   - Homeostasis of red blood cells; describe how clinical conditions such as hypoxia can lead to polycythaemia
   - Mechanism of action of targeted therapy in myeloproliferative neoplasms (e.g. imatinib)
Clinical and Communication Skills 
   - History from a patient with polycythaemia; consider secondary polycythaemia; recognise symptoms of hyperviscosity
   - Examine the cardiovascular and respiratory system and consider secondary polycythaemia; examine the abdomen (document spleen size)
   - Differential diagnosis of polycythaemia and causes of secondary polycythaemia
   - Differential diagnosis of thrombocytosis; differential diagnosis of granulocytosis
   - Interpret full blood count and blood film report in a patient with a myeloproliferative neoplasm: polycythaemia vera, essential thrombocythemia, primary myelofibrosis and chronic myeloid leukaemia
   - Indications for assessing erythropoietin level , molecular tests (JAK2 and BCR-ABL), and bone marrow biopsy
   - Management of myeloproliferative neoplasms
   - Prognosis and complications of myeloproliferative neoplasms: thrombosis, progression to myelofibrosis and transformation to acute leukaemia
Personal and Professional Skills 
   - Equipoise and enrolling patients in research
   - Understand the burden of chronic disease on patient, his/hertheir family/whānau and the healthcare system
Hauora Māori 
   - Appropriate engagement and consultation with whānau
   - Consideration of access to cultural/spiritual support for patient and whānau
Population Health 
   - Epidemiology of myeloproliferative neoplasms
Conditions to be considered relating to this scenario
Common
chronic obstructive pulmonary disease, obstructive sleep apnoea, polycythaemia vera, obesity hypoventilation syndrome
Less common but 'important not to miss'
renal cell carcinoma, hepatocellular carcinoma, cyanotic heart disease, altitude sickness
Uncommon
high oxygen affinity haemoglobinopathy, methaemoglobinaemia