Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Blood and Lymph > Sickle Cell > Flashcards

Sickle Cell Flashcards

1
Q

What CBC results will be seen bc of the hemolytic anemia?

A

anemia, high retic count, increased RDW, sometimes increased WBCs and platelets

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

What is normal progression of hemoglobins on the beta chain from fetus to adult?

A

epsilon, gamma, delta, beta

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

What is Hydroxyurea?

A

an oral chemo agent

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

_____ occurs in a person with one sickle cell gene and one normal gene.

A

Sickle cell trait

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

_____ occurs in persons who have two abnormal β-globin genes; at least one of these genes has the sickle mutation.

A

Sickle cell disease

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

What is the treatment for iron overload?

A

a chelation agent

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

What is the acute, severe swelling of the hands and feet, usually in sickle cell infants called?

A

hand-foot syndrome

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

How is sickle cell inherited?

A

auto. recessive

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

Name 3 complications of hemolysis in sickle cell.

A

1) aplastic crisis 2) growth retardation or delay 3) bilirubin gallstones

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

What can occur after several transfusions?

A

iron overload

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

What findings will be seen in Sickle Cell on the peripheral smear?

A

sickle forms, schistocytes, polychromasia

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

What are the 3 major treatments of sickle cell?

A

1) bone marrow transplant 2) Hydroxyurea therapy 3) transfusion

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

Where are the hemoglobin beta genes located?

A

chromosome 11

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

What does normal hemoglobin A2 consist of?

A

alpha2-delta2

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

What is the predominant hemoglobin after age 2?

A

HbA

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

When deoxygenated, sickle hemoglobin polymerizes into _____ which distort the shape of the RBC into a “sickle form.”

A

14-strand helical fibers

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

How long do sickle RBCs last in circulation?

A

20 days

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

Retinal vessel damage can lead to ____ and ____.

A

retinal detachment; blindness

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

Sickle cell disease occurs in persons who have two abnormal β-globin genes; at least one of these genes has _____.

A

the sickle mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is normal progression of hemoglobins on the alpha chain from fetus to adult?

A

zeta, alpha

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is acute chest syndrome?

A

sickle RBCs become trapped in the lungs- damage endothelium- fluid leaks in- can’t breathe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What abnormal chemistry findings will be seen in sickle cell anemia?

A

increased bilirubin, LDH, AST

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Why do skin ulcers occur in sickle cell anemic?

A

microvascular ischemia and poor healing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

When large numbers of sickle RBCs become abruptly trapped in the spleen, causing severe anemia and shock, ______ has occurred.

A

splenic sequestration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Nearly all patients with sickle cell anemia (HbSS) chronically occlude the spleen’s microcirculation, resulting in “autoinfarction” (destruction) of the spleen by the age of ___.

A

5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the beta globin genes in sickle cell disease?

A

HbSS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

In what locations can sickles be formed in sickle cell train?

A

renal medulla

25
Q

How is sickle cell diagnosed?

A

gel electrophoresis (separation) of the hemoglobins

26
Q

What is sickle cell crisis?

A

when a stressor, coupled with BV damage and constriction, causes sickling and pain

26
Q

What does normal hemoglobin A1 consist of?

A

alpha2-beta2

28
Q

Name locations that sickle cells tend to adhere and cause vascular occlusion.

A

1) spleen 2) CNS 3) lungs 4) kidney 5) retina 6) femoral and humeral head 7) ankle skin (ulcers)

29
Q

What is strain on the R side of the heart called?

A

cor pulmonale

31
Q

What is splenic sequestration?

A

when large numbers of sickle RBCs become abruptly trapped in the spleen, causing severe anemia and shock

31
Q

Why is Hydroxyurea used to treat sickle cell pts?

A

fetal hemoglobin produced interferes with sickle hemoglobin polymerization

31
Q

What does normal hemoglobin F consist of?

A

alpha2-gamma2

32
Q

What is priapism?

A

sustained, painful erections

33
Q

What is hand-foot syndrome?

A

acute, severe swelling of the hands and feet, usually in sickle cell infants

33
Q

What is a limitation for bone transplants in sickle cell patients?

A

finding a match- less than 20% have a donor avail

34
Q

What is the average lifespan of a sickle cell pt?

A

50-60

35
Q

How much HbF is seen at a normal birth?

A

60-90%

36
Q

What does Hydroxyurea do?

A

it induces production of fetal hemoglobin in persons older than 12 mos

38
Q

Sickled RBCs cause damage to the lung microvasculature, leading to _____.

A

pulmonary arterial hypertension (PAH)

39
Q

Why do sickle cells have a propensity to adhere to one another?

A

membrane injury, retention of surface adhesion molecules

40
Q

How much HbA is seen at a normal birth?

A

10-40%

42
Q

What are the beta globin genes in sickle cell trait?

A

HbAS

44
Q

How are the kidneys affected by sickle cells?

A

tubules damaged, leading to inability to concentrate urine (to prevent dehydration); glomerular damage

47
Q

When ______, sickle hemoglobin polymerizes into 14-strand helical fibers which distort the shape of the RBC into a “sickle form.”

A

deoxygenated

49
Q

Sickle cell disease occurs in persons who have two abnormal _____; at least one of these genes has the sickle mutation.

A

β-globin genes

50
Q

What is aplastic crisis?

A

a sudden drop in hemoglobin from compromised bone marrow

51
Q

When are the RBCs more likely to sickle?

A

hypoxia, dehydration, inflammation, infection, or other stresses

52
Q

What causes aplastic crisis in children?

A

Parvovirus B19

53
Q

What is the predominant hemoglobin in sickle cell trait?

A

hemoglobin A

54
Q

In what instances can sickles be formed in sickle cell trait?

A

exertion at altitude coupled with dehydration

55
Q

How do bone infactions occur in sickle cell?

A

chronic ischemia leads to necrosis

55
Q

How much HbA2 is seen at a normal birth?

A

less than 1%

56
Q

Nearly all patients with sickle cell anemia (HbSS) chronically occlude the spleen’s microcirculation, resulting in “_______” (destruction) of the spleen by the age of 5.

A

autoinfarction

57
Q

Nearly all patients with sickle cell anemia (HbSS) chronically occlude the spleen’s ______, resulting in “autoinfarction” (destruction) of the spleen by the age of 5.

A

microcirculation

58
Q

What can potentially cure sickle cell disease?

A

bone marrow transplant

59
Q

What are the requirements for a bone marrow transplant donor?

A

HLA-matched, full sibling, unaffected

60
Q

Where does iron overload affect the body?

A

liver and heart

61
Q

Sickle cell trait occurs in a person with _____.

A

one sickle cell gene and one normal gene

62
Q

What are the 3 main sickle cell clinical manifestations?

A

1) chronic hemolytic anemia 2) chronic RBC adhesion/vascular occlusion 3) acute RBC adhesion/vascular occulusion

63
Q

What is a risk patients with sickle cell face because of RBCs damaging the CNS?

A

stroke

64
Q

Where are the hemoglobin alpha genes located?

A

chromosome 16

65
Q

What finding is characteristic of aplastic crisis?

A

low retic count

Blood and Lymph (24 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions