PV Flashcards

1
Q

What is the relationship between elevated Hct levels (45%-50%) and cardiovascular death?

A

A 4-fold higher rate of cardiovascular death and major thrombosis is associated with elevated Hct levels between 45%-50% compared to Hct levels of <45%.

This indicates that maintaining Hct levels below 45% is crucial for reducing cardiovascular risks.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What WBC count is associated with an increased risk of thrombosis?

A

WBC counts >11 x 10^9/L are associated with an increased risk of thrombosis.

This finding was based on a multivariable time-dependent analysis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the golden takeaway regarding strict HCT control?

A

Any hematocrit level above 45% needs action.

The recommendation emphasizes that even levels slightly above 45% are considered problematic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Define Polycythemia Vera (PV).

A

PV is a rare, chronic blood cancer where the body makes too many red blood cells, white blood cells, and platelets.

PV is part of a group of diseases called myeloproliferative neoplasms (MPNs).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What effect do too many red blood cells have on blood flow?

A

Too many red blood cells can cause the blood to thicken, which affects normal blood flow through arteries and veins.

This thickening can lead to various complications, including thrombosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is hematocrit (HCT)?

A

HCT is the percentage by volume of red cells in the blood.

Blood comprises red blood cells, white blood cells, and platelets, which together make up about 45% of blood volume.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is phlebotomy?

A

Phlebotomy is a procedure in which blood is taken from a vein, usually for laboratory testing or to remove extra red blood cells.

It is also referred to as a blood draw or venipuncture.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the function of hemoglobin (Hb)?

A

Hb is the protein in red blood cells responsible for the delivery of oxygen to tissues.

Sufficient hemoglobin levels are necessary to ensure adequate tissue oxygenation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Fill in the blank: Hemoglobin levels in whole blood are expressed in _______.

A

grams per deciliter (g/dl)

Maintaining proper hemoglobin levels is crucial for health.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is phlebotomy?

A

A procedure where a needle is used to take blood from a vein, usually for testing or medical treatment

Also known as a blood draw or venipuncture. It can be used to remove excess red blood cells in certain conditions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is hemoglobin (Hb)?

A

A protein in red blood cells that carries oxygen throughout the body

Important for overall health as tissues need enough oxygen to function properly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the function of the spleen?

A

Filters blood, stores certain blood cells, and produces white blood cells that fight infections

Located under the left ribs, near the stomach.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What does hematocrit (HCT) measure?

A

The percentage of blood made up of red blood cells

Normally, red blood cells make up about 45% of total blood volume.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is thrombosis?

A

A condition where a blood clot forms inside a blood vessel or the heart

Can lead to blocked blood flow, stroke, or heart attack.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is thrombocytopenia?

A

A condition characterized by having too few platelets

Can result in easy bruising or excessive bleeding.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the mechanism of action for Jakafi® (Ruxolitinib)?

A

Blocks specific proteins (JAK1 and JAK2) involved in inflammation and blood cell production

Helps reduce abnormal blood cell growth and inflammation.

17
Q

What were the goals of the Comfort Studies I & II?

A

To evaluate how well Jakafi works for myelofibrosis patients compared to a placebo or best available therapy

Aimed to assess spleen shrinkage and symptom reduction.

18
Q

What was a key finding from the Comfort Studies I & II?

A

About 28% of patients saw their spleen shrink by 35% or more

Jakafi improved symptoms like tiredness, night sweats, and itching in 46% of patients.

19
Q

What does the term ‘Post Hoc Analysis’ refer to in the context of the Comfort Studies?

A

Re-examining the original study results for additional insights

Aimed to find further useful information beyond initial findings.

20
Q

What was a finding from the JUMP Study regarding Jakafi?

A

About 60% of patients had some spleen size reduction

Symptoms improved for most patients, with a safety profile similar to clinical trials.

21
Q

What did the CYTO-PV analysis find regarding white blood cell counts?

A

A 4-fold increase in the risk of major thrombosis among patients with white blood cell counts above 11.

22
Q

What percentage of patients receiving ruxolitinib achieved the combined primary endpoint in the RESPONSE study?

A

23%

Compared to less than 1% of those who received best available therapy.

23
Q

What percentage of patients receiving ruxolitinib achieved complete hematologic remission?

A

24%

Those patients had a 55% probability of maintaining it at 5 years.

24
Q

What type of trial was the MAJIC-PV study?

A

Open-label, phase 2, randomized, controlled trial.

25
What was the primary endpoint of the MAJIC-PV study?
Complete response rate within 12 months as defined by ELN criteria.
26
What criteria define a complete response in the MAJIC-PV study?
Hematocrit less than 45% without phlebotomy for 3 months; platelets less than or equal to 400; white blood cell count less than or equal to 10; and normal spleen size.
27
What percentage of patients receiving ruxolitinib achieved complete response within 12 months?
43% ## Footnote Compared to 26% of patients receiving best available therapy.
28
What reduction in risk of an event did ruxolitinib demonstrate in the MAJIC-PV study?
42% reduction in the risk of an event.
29
What was the reduction in the risk of major thrombosis with ruxolitinib in the MAJIC-PV study?
44% reduction.
30
How many phlebotomies did patients in the best-available-therapy arm undergo compared to those in the ruxolitinib arm?
307 vs. 83.
31
What are the warning signs indicating inadequate treatment with hydroxyurea?
Hematocrit levels between 45% and 50%, white blood cell counts above 11, or needing more than 4 phlebotomies a year.
32
What is the desired hematocrit level for managing polycythemia vera?
Less than 45%.
33
What should be considered if a patient has needed 2 or more phlebotomies in the last 6 months?
Discuss a change in treatment.
34
What is the significance of controlling hematocrit and white blood cell count in treatment?
There is a benefit in reducing the risk of thrombosis.