Chronic Myeloproliferative Neoplasms

Question 1

MC of all chronic myeloproliferative neoplasms

Answer 1

Polycythemia Vera

Question 2

Only symptom that distinguishes PV from other causes of erythrocytosis

Answer 2

Aquagenic pruritus

Question 3

Catastrophic complication of complete obstruction of the hepatic veins of patients with PV (commonly in young women)

Answer 3

Intraabdominal Venous Thrombosis

Question 4

DDx for Microcytic Erythrocytosis

Answer 4

B thalassemia trait
Hypoxic erythrocytosis
Polycythemia vera

Question 5

Hgb and Hct goals in PV to avoid thrombotic complications

Answer 5

Males: Hgb = 140; Hct <45%
Females: Hgb = 120; Hct <42%

Question 6

Can establish diagnosis of PV

Answer 6

JAK2 V617F Assay

BMA biopsy no specific diagnostic info

Question 7

Only curative therapy for Primary Myelofibrosis (MPF)

Answer 7

Allogenic BM transplant (esp in younger patients)

Question 8

Complication of thrombocytosis consisting erythema + burning + pain in the extremities

Answer 8

Erythromelalgia

Tx: NSAIDs