Polycythemia

- Hematocrit > 55% (and elevated Hb) due to increase in number of RBC “absolute polycythemia” or
decrease in plasma volume “relative polycythemia”
- Signs

General - Plethora/ Peripheral cyanosis

- Cushing appearance/Virilised appearance

Vitals - Tachycardia- thickened viscosity

- Hypertension – phaeochromocytoma, increased plasma volume “relative
polycythemia”, thickened viscosity

Hands - Cool hands (cyanosis)

- Darkened creases of palm
- Palmar erythema
- CR prolonged- thickened viscosity
- Nicotine staining – smoking
- Peripheral cyanosis
- Arthropathy/gouty changes – (increased urea formation)

Arms - Scratch marks – PCRV due to basophil histamine release

- Briuse/petichae- often have clotting disorder – Thrombophilia from JAK 2
mutation

Face - General- Plethoric

- Eyes- Scleral injection/hemorrhages and engorged retinal vessels (fundoscopy)
- Mouth- Dehydration (relative polycythemia cause), peripheral/central cyanosis
(underlying heart disease or chronic lung disease), petichae (clotting disorder)

Chest - Cardiovascular- CF (JVP increased, apex displaced), systolic flow murmur, murmur
for cyanotic heart disease
- Respiratory- signs of chronic lung disease, infections

Abdomen - Masses/ascites
- Splenomegaly- occurs in 80% of cases of PRV but does NOT occur with other
causes of polycythemia
- Hepatomegaly
- Kidney palpation – polycystic kidney disease, hydro nephrosis, renal carcinoma
- Bi-manual- Uterus palpation for women – uterine fibroma

Legs - Temperature- slugglish

 - Edema- viscous
- Scratch marks – PRV
- Gout/ Arthropathy- increased general catabolism because of increased proliferation
of RBC)
- CR
- Peripheral cyanosis
- Signs of peripheral vascular disease – ulcers, dry skin– due to hyper viscosity
- Peripheral pulse- decreased – slugglish

CNS - Cerebellar signs due to presence of cerebellar haemangioblastoma, very rare cause
of secondary polycythemia
- CNS for signs of stroke due to thrombosis

Investigations

- FBC
- Peripheral blood smear
- JAK 2 mutation – genotyping
- ESR- check for plasma viscosity
- LFTS- to check for increased LDH – increased from increased B/D
- EPO – secondary causes
- Clotting profile