Myeloproliferative Syndromes

Question 1

What are the phases of polycythemia vera?

Answer 1

• latent phase - relatively asymptomatic
• proliferative phase: hyperviscosity, hypermetabolic symptoms (fever, weight loss, sweating), or thrombosis
• spent phase: 20% of pts with PV, fibrosis enters the bone marrow, driving hematopoeitic activity into the liver and spleen -> HSM

Question 2

What are the common signs/symptoms of PV?

Answer 2

• confusion, TIA like symptoms, tinnitus, blurred vision, headache (50% of pts) - occurs in all types of erythrocytosis
• generalized pruritis, worse after bathing; erythromelalgia (burning in palms and soles, ?platelet activation), hypermetabolic symptoms (fever, weight loss, sweating) = occurs in myeloproliferative diseases
• may have plethora and splenomegaly on exam
• thrombotic and bleeding symptoms are common