Background
- Chronic myeloproliferative disorder
- Abnormal proliferation is seen in all 3 cell lines
- Typically in elderly
- Can be asymptomatic or symptomatic
Clinical Presentation
- Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
- Suspected with any combination of the following: [1]
- Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
- Normal oxygen saturation
- Bleeding complications
- Portal vein thrombosis
- Splenomegaly
- Plethora
- Pruritus after bathing
DDx
- Essential thrombocytosis (ET)
- Chronic myelogenous leukemia (CML)
- Leukemoid reactions
- Hypoxia
Labs
- CBC
- Chem 7
- ESR
- EPO
- Bone Marrow Bx sometimes needed
Treatment
- Phlebotomy
- ASA 81 mg
- Myelosuppressive agents
Also See
Sources
http://emedicine.medscape.com/article/205114-differential