Proliferative disorder where bone marrow inappropriately produces RBCs, non-EPO dependent
Associated w/JAK2 mutation
Secondary erythrocytosis is caused by elevated EPO from tumors (e.g. RCC, HCC, fibroids) chronic hypoxemia (e.g. OSA, severe COPD, other), meds (e.g. testosterone). These processes don't lead to hypercoaguable states.
Asx until hgb significantly elevated
Itching exacerbated by hot shower
Sx related to sludging→ vague abd pain, sob, wt loss, head ache, TIA, DVT, stroke
Higher risk for arterial events (in particular if no other RFs)
Higher risk for DVTs, consider in particular if unusual locations (mensenteric, cerebral veins, etc) and/or no other RFs