Polycythemia Vera

Question 1

How will you define polycythemia vera?

Answer 1

Increased RBCs and panmyelosis

Question 2

WHat is the mutation in PCV?

Answer 2

JAK2 tyrosine kinase (valine to phenylalanine at residue 617)

Question 3

Erythropoietin levels are ______ (high/low) in PCV

Answer 3

low

Question 4

Hematocrit is _________ (increased/decreased) in PCV

Answer 4

increased (60%)

Question 5

Platelets are _________ (increased/decreased) in PCVN

Answer 5

increased

Question 6

Is there marrow fibrosis present in polycythemia vera?

Answer 6

Yes

Question 7

What will hapeen if there are two copies of mutation present in PCV?

Answer 7

Increased RBCs, WBCs etc
Splenomegaly
Symptomatic pruritis
Spent phase risk

Question 8

What are the findings in bone marrow?

Answer 8

Hypercellularity with residual fat
Increase in precursors of every cell of myeloid origin
Increased reticulin deposition

Question 9

What is the cause of organomegaly in early and late phase of PCV?

Answer 9

Early -mild - congestion causing organomegaly

Late - severe - extramedullary hematopoiesis

Question 10

What do we find in the peripheral blood smear of PCV patients?

Answer 10

Basophils
Large platelets (defective)

Question 11

Why does severe organomegaly occurs in late phase?

Answer 11

in late phase, PCV has been changed into spent phase, this means marrow is fibrosed leading to EMH in other organs like spleen

Question 12

There is 1% chance PCV can transform into _______ (AML/ALL)

Answer 12

AML

Question 13

Onset of PCV is _______ (acute/chronic)

Answer 13

chronic

Question 14

PCV is most common in adults of _________ (early young/late middle) age

Answer 14

late middle age

Question 15

What are the changes in the color of skin in PCV?

Answer 15

plethoric

cyanotic

Question 16

Increased hematocrit results in an abnormal blood flow, causing the veins to become distended. Blood ________ and deoxygenation occurs causing the color of skin to change to plethoric and cyanotic.

Answer 16

stagnation

Question 17

What are the symptoms in PCV due to basophilia?

Answer 17

Intense pruritis

Peptic ulcers

Question 18

How do basophils cause symptoms in PCV?

Answer 18

hISTAMINE IS RELEASED FROM BASOPHILS

Question 19

What are the symptoms due to high hematocrit?

Answer 19

headache, dizziness, HTN, GI symptoms

Question 20

What are the other symptoms of PCV?

Answer 20

Hyperuricemia and gout (due to high cell turnover)

Thrombosis and bleeding

Question 21

what are the thrombotic complications of PCV?

Answer 21

DVT
MI
Stroke
Budd Chiari Syndrome
Bowel infarction

Question 22

Thrombosis may occur ________ (earlier/later) than appearance of the typical hemotological findings

Answer 22

earlier

Question 23

Lab findings of PCV?
Hb
Hct
Iron
WBC
Platelets

Answer 23

Hb - 14-28gm/dL
Hct - 60%
Iron - deficient
WBC - 12,000-50,000
Platelets - 500,000

Question 24

What is the tx of PCV?

Answer 24

phlebotomy

jak2 inhibitors

Question 25

during PCV tx, spent phase occurs in some patients in ____ years

Answer 25

10