Myeloproliferative Disorders

Question 1

Myeloproliferative

Answer 1

Overproduction of cells in the bone marrow
Chronic myelogenous leukemia
Polycythemia vera
Myelofibrosis
Essential thrombocystosis

Question 2

Polycythemia vera (PV)

Answer 2

Characterized by elevated H/H

Elevated red cell mass

Question 3

3 causes of polycythemia

Answer 3

Appropriately elevated EPO hypoxemia
Innapropriately elevated EPO (carcinoma)
Germline and somatic mutation (Pvera)

Question 4

PV occurs most frequently in ___ over the age of ____.

Answer 4

Men over the age of 60.

most common 70 - 79

Question 5

PV survival

Answer 5

untreated: 6 - 18 months
treated: >10 yrs

Question 6

PV Symptoms

Answer 6

HA, weakness, dizziness, sweating.
Pruritis post-bathing***
Erythromelalgia (burning in feet/hands)
Thrombotic events
GI discomfort

Question 7

PV lab findings

Answer 7

H/H > 18.5/56 men, 16.5/50 women
WBC: > 10.5
Platelets > 450k
Elevated LDH
JAK-2 mutation
abnormal serum epo
Absent iron storage in marrow

Question 8

PV exam findings

Answer 8

splenomegaly
facial plethora
hepatomegaly
injection of conjunctiva
excoriation of skin
gouty arthritis and tophi

Question 9

PV Tx

Answer 9

Phlebotomy to keep HCT < 45 or 42 (fem)
Myelosuppressive agents
Antihistamines (pruritis)
Allopurinol (gout)
Aspirin (erythromelalgia)

Question 10

Myelosuppressive agents

Answer 10

Hydroxyurea

Alpha interferon

Question 11

What can PV progress to?

Answer 11

Myelofibrosis and Acute Lukemia

Question 12

Myelofibrosis (PMF)

Answer 12

Marrow is replaced with collagenous connective tissue fibers.

Question 13

Myelofibrosis facts

Answer 13

Least frequent of chronic myeloproliferative disorders
Middle aged and elderly (median age 67)
Rare in childhood
More often in men than women

Question 14

Myelofibrosis Signs

Answer 14

Severe fatigue *
LUQ Pain * radiating to L shoulder
Early satiety
Weight loss
low grade fever/night sweats
Spleno/hepatomegaly

Question 15

Myelofibrosis leads to?

Answer 15

Acute leukemia
Infection
Bleeding
Portal HTN
Vascular complications

Question 16

Myelofibrosis morphology

Answer 16

Tear drop RBC’s
Poik
Anisocytosis

Question 17

PMF lab findings

Answer 17

WBC can be low or high
Platelets can be low or high
Elevated alkaline phosphotase
Elevated LDH
Elevated Uric acid
Mat have JAK-2 mutation

Question 18

PMF Tx

Answer 18

Stem cell transplant
Hydroxyurea
Chemotherapy
JAK-2 inhibitors
Splenectomy
Radiation

Question 19

Extramedullary Hematopoesis

Answer 19

Caused by myeloproliferative disorder
Other organs than the marrow producing RBC’s
Can occur in almost any organ, rendering it useless
Treated with radiation

Question 20

Is PMF Ph (philadelphia chromosome) +?

Answer 20

Nope, its negative. boom.

Question 21

Essential Thrombocytosis (ET)

Answer 21

Overproduction of platelets
Abnormal platelet count
Isolated thrombocytosis
Occurs in ages over 50

Question 22

ET lab findings

Answer 22

Elevated platelet count, but normal values
Elevated megakaryocytes
JAK-2 present in many cases

Question 23

ET symptoms

Answer 23

HA, lightheadedness, visual changes
Numbness, tingling, burning
splenomegaly
Thrombosis

Question 24

ET Tx

Answer 24

Low dose aspirin

Hydryxyurea: keep platelets below 500,000

Question 25

ET can progress to…

Answer 25

Myelofibrosis and acute leukemia

massive splenomegaly