Polycythaemia Rubra Vera
Definition
Clonal d/o
Dx of exclusion
NOTE: Does not exist in HH unless there is another dx
Aetiology
Secondary
Investigations
Bedside: Spirometry
Bloods: Serum EPO
Imaging: CXR, Renal US
Others: JAK-2 mutation (in ET, sometimes in PRV), BM aspirate (cytogenetics)
Management
Complications
Source
Prof Kim Rooney 2014
Clonal d/o
Dx of exclusion
NOTE: Does not exist in HH unless there is another dx
Aetiology
Secondary
- Sleep apnoea
- RCC
- Smoking
- Dehydration
- Exogenous erythropoietin
- High altitude acclimatisation
Investigations
Bedside: Spirometry
Bloods: Serum EPO
Imaging: CXR, Renal US
Others: JAK-2 mutation (in ET, sometimes in PRV), BM aspirate (cytogenetics)
Management
- Isovolaemic venesection (give N saline wth venesection to avoid haemoconcentration)
- Aim for Hb 160, MCV 65 (Fe deficient)
Complications
- Thrombosis (viscosity)
- Haemorrhage (bv hypoxemic due sludge)
- Myelofibrosis (see left shift in erythro-progenitor cells)
- AML
Source
Prof Kim Rooney 2014