iatroX Rounds #34
The clues
- 1
A 64-year-old man presents with intense, generalized skin itching that is particularly severe after taking a warm bath or shower.
- 2
He also reports a persistent sensation of fullness in his left upper abdomen, along with recurring headaches and occasional blurred vision.
- 3
The patient describes a history of gout and mentions episodes of burning pain in his toes and fingertips, which become red and warm to the touch.
- 4
On examination, he has a noticeably ruddy, plethoric complexion, and the tip of the spleen is palpable 4 cm below the left costal margin.
- 5
Initial blood tests show a haemoglobin level of 192 g/L and a haematocrit of 0.59, while the serum erythropoietin level is found to be suppressed.
- 6
Molecular analysis of peripheral blood reveals a V617F point mutation in the Janus kinase 2 gene.
Polycythaemia vera is a chronic myeloproliferative neoplasm characterized by the autonomous overproduction of red blood cells. The presentation is driven by hyperviscosity, histamine release causing aquagenic pruritus, and microvascular complications like erythromelalgia.
- Aquagenic pruritus
- Splenomegaly
- Erythromelalgia
- Raised haematocrit with low erythropoietin
for education and entertainment. not medical advice.