Screening 4

Question 1

polycythemia aka

Answer 1

erythrocytosis

Question 2

Polycythemia occurs when there are too many

Answer 2

RBCs

Question 3

In polycythemia, increased concentration of hemoglobin leads to

Answer 3

• increased blood volume

- increased blood viscosity

Question 4

Increased blood volume and viscosity limits blood’s ability to

Answer 4

flow easily

Question 5

What are the two forms of polycythemia?

Answer 5

• primary

- secondary

Question 6

primary polycythemia aka

Answer 6

polycythemia vera

Question 7

What is primary polycythemia?

Answer 7

rare bone marrow neoplasm

Question 8

Primary polycythemia and RBC/WBC

Answer 8

• decreased RBC production
• increased # of RBCs
• usu causes excess WBC and platelet production

Question 9

What happens to blood in the capillaries with polycythemia and what outward sign does this produce?

Answer 9

• viscous blood plugs capillaries

- get “hot bath sign”

Question 10

Secondary polycythemia results from

Answer 10

decreased oxygen to the tissues

Question 11

Secondary polycythemia may be due to (environmental)

Answer 11

• living at high altitudes
• smoking
• radiation
• chronic heart/lung disorders

Question 12

Initial s/s of polycythemia

Answer 12

• usually vague

- watch for increased BP and hematocrit levels

Question 13

Why are BP and hematocrit levels increased with polycythemia?

Answer 13

due to increased blood viscosity

Question 14

What are the 2 features of sickle cell anemia?

Answer 14

• hemolytic anemia

- vasoocclusion

Question 15

What genetic mutation is seen in sickle cell anemia?

Answer 15

RBCs contain an abnormal hemoglobin S

Question 16

What does abnormal hemoglobin S cause?

Answer 16

• aggregation into long beta chains

- alters shape of cell

Question 17

When hemoglobin S is exposed to low concentrations of O2, what happens?

Answer 17

develops sickle appearance instead off a biconcave disc

Question 18

In addition to the shape change seen with sickle cell anemia, what else happens to the RBC? What does this cause?

Answer 18

• RBCs are highly fragile

- leads to serious anemia

Question 19

What type of disease is sickle cell anemia?

Answer 19

• inherited

- autosomal recessive

Question 20

sickle cell anemia: autosomal recessive means

Answer 20

both copies of the gene in each cell have mutations

Question 21

Sickle cell anemia is the most common ________

Answer 21

inherited blood disorder in the US

Question 22

sickle cell anemia: African Americans

Answer 22

1 in 500

Question 23

sickle cell anemia: Hispanic Americans

Answer 23

1 in 1000

Question 24

manifestation of sickle cell anemia is characterized by

Answer 24

• a series of “crises/episodes” of acute manifestations of the disease
• range in severity

Question 25

sickle cell anemia: recurrent episodes result in

Answer 25

progressive damage to most organs

Question 26

What medical issues are frequent and severe with pts who have sickle cell anemia?

Answer 26

• CVAs

- cognitive impairment

Question 27

The hot bath sign causes:

Answer 27

Intolerable itching

Question 28

Clinical signs of polycythemia are usually vague initially: what are some common things that happen?

Answer 28

• Decreased mental acuity
• Blurred vision
• Headaches
• etc.

Question 29

With polycythemia, what values should you monitor closely and why?

Answer 29

monitor BP and hematocrit because of increased blood viscosity