Makindo Medical Notes"One small step for man, one large step for Makindo" |
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Cause | Clinical Features | Clinical Tests | Management |
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Primary Polycythaemia (Polycythaemia Vera) |
- Symptoms of hyperviscosity: headache, dizziness, blurred vision, pruritus (often after a hot shower).
- Splenomegaly, thrombosis, and erythromelalgia (burning pain in hands/feet with redness). |
- Full blood count (FBC): Raised red cell mass, elevated haematocrit.
- JAK2 mutation test: Positive in >95% of cases. - Serum erythropoietin (EPO): Typically low. |
- Regular venesection (phlebotomy) to reduce haematocrit.
- Low-dose aspirin to reduce thrombosis risk. - Cytoreductive therapy (e.g., hydroxyurea) in high-risk patients. |
Secondary Polycythaemia (Hypoxia-Driven) |
- Symptoms related to underlying hypoxia (e.g., chronic lung disease, obstructive sleep apnoea, high altitude).
- Cyanosis, dyspnoea, or clubbing may be present. |
- FBC: Elevated red cell mass and haematocrit.
- Arterial blood gas (ABG): Hypoxia and elevated pCO2 in chronic lung disease. - Serum EPO: Elevated due to hypoxia-driven erythropoiesis. |
- Treat underlying hypoxic condition (e.g., COPD, sleep apnoea).
- Oxygen therapy for chronic hypoxia. - Venesection may be considered if symptomatic or haematocrit is very high. |
Secondary Polycythaemia (EPO-Producing Tumours) |
- Fatigue, weight loss, or night sweats due to malignancy.
- Symptoms of polycythaemia (e.g., dizziness, headache) may be present. - Possible palpable mass (e.g., renal tumour). |
- FBC: Raised red cell mass and haematocrit.
- Serum EPO: Elevated. - Imaging: CT/MRI to locate tumour (e.g., renal cell carcinoma, hepatocellular carcinoma). |
- Treat underlying malignancy (e.g., surgery for renal cell carcinoma).
- Chemotherapy or radiotherapy for tumour management. - Venesection for symptomatic relief if polycythaemia persists. |
Relative Polycythaemia (Dehydration) |
- Symptoms of dehydration: dry mucous membranes, reduced skin turgor, hypotension.
- Dizziness, fatigue, and increased thirst. |
- FBC: Raised haematocrit but normal red cell mass.
- Serum electrolytes: May show hypernatraemia due to dehydration. - Urinalysis: Concentrated urine with high specific gravity. |
- Rehydration with oral or intravenous fluids.
- Correct underlying cause of dehydration (e.g., diarrhoea, vomiting, diuretics). - Monitor fluid status and electrolytes. |
Relative Polycythaemia (Gaisböck Syndrome) |
- Common in middle-aged men with obesity, hypertension, and stress.
- No true increase in red cell mass, but relative increase due to reduced plasma volume. |
- FBC: Elevated haematocrit, normal red cell mass.
- Serum EPO: Normal. - Blood pressure and cardiovascular risk assessment. |
- Lifestyle modifications: Weight loss, control of hypertension.
- Venesection may be performed in symptomatic cases. - Aspirin for cardiovascular risk reduction. |
Drug-Induced Polycythaemia (e.g., Testosterone Therapy) |
- History of anabolic steroid or testosterone use.
- Symptoms may include fatigue, headache, or visual disturbances. |
- FBC: Elevated haematocrit.
- Serum EPO: Normal or slightly elevated. - Drug history: Confirm use of erythropoietic drugs or steroids. |
- Discontinue or reduce the dose of the offending drug.
- Regular monitoring of haematocrit and FBC. - Venesection if haematocrit remains high after cessation. |