EM Hema 1: Polycythemia

Question 1

classic presentation of PCV

Answer 1

Pruritus when exposed to hot water and early satiety from splenomegaly

plethoric facies and ruddy skin
distal cyanosis and clubbing from microemboli

Question 2

criteria for polycythemia

Answer 2

HEMOGLOBIN

males: >185 g/L
females: >165 g/L

HEMATOCRIT

m: >52%
f: >48%

Question 3

remarks on hyperviscosity syndrome

Answer 3

life-threatening complication of polycythemia

triad of bleeding, visual disturbances, and focal neurologic deficits caused by thrombosis and microhemorrhage

Question 4

symptoms of hyperviscocity syndrome

Answer 4

initially: fatigue, abd pain, headache, blurry vision
m/c: altered mental status

Question 5

cut-off values associated with clinically significant hyperviscosity

Answer 5

hct >60%

wbc >100,000/mm3

Question 6

treament of PCV

Answer 6

Mainstay:
low-dose aspirin and phlebotomy to a hct of 45%

if with hyperviscocity syndrome:
aggressive iV hydration
early hematology consult

Question 7

Hyperviscosity syndrome: if coma is present and the diagnosis is established, a temporaizing measure can be:

Answer 7

a 2-unit (1000-mL) phlebotomy with concomitant volume replacement using 2-3 L of normal saline

Question 8

other pharmacotherapy for polycythemia

Answer 8

hydroxyurea
interferon-a
systemic anticoagulation
JAK1/JAK2 inhibitor

Question 9

example of JAK1/JAK2 inhibitor

Answer 9

ruxolitinib