Brainscape's Knowledge GenomeTM

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


See full index

Hematology I > Hemoglobinopathies > Flashcards

Hemoglobinopathies Flashcards

1
Q
  • result due to differences in the arrangement of amino acids in the polypeptide chains
  • alphabetically designated
  • qualitative
A

Hemoglobinopathies

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

prevalence of hemoglobinopathies (worldwide)

A

Hb S, Hb E, Hb C

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

Severity of hemoglobinopathies

A

HbSS, Hb SC, Hb CC

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

hemoglobinopathies are differentiated from one another by:

A
  • solubility

- mobility in an electrophoretic field

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
  • screening test for detection of sickling hemoglobin

- not specific for HbS

A

Sodium Dithionite (Hydrosulfite) Tube test

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

principle of sodium dithionite tube test

A
  • red cells are lysed by saponin

- sodium dithionite binds & removes oxygen from the test environment

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

False (+) result of sodium dithionite tube test

A
  • hyperlipidemia
  • rare hemoglobinopathies
  • too much blood added to solution
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

false (-) result of sodium dithionite tube test

A
  • infants less than 6 months of age

- decreases hematocrit/RBC

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

under deoxygenated state, Hb S causes formation of ____

A

sickle cells

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

found in sickle cell trait, reported as positive

A

“holly leaf”

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

a reducing agent that deoxygenates hemoglobin

A

sodium metabilsufite

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

negative result of 2% sodium metabisulfite test

A

normal RBCs/slightly crenated

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

hemoglobin molecules have a net negative charge at____

A

alkaline pH

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

hemoglobin migrate towards the _____

A

anode (positive)

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

fastest in cellulose acetate

A

Hb H & Hb I

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

slowest in cellulose acetate

A

Hb C, E, O & A2

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

pH of cellulose acetate

A

8.4-8.6

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

buffer used in cellulose acetate membrane

A

tris-EDTA-boric acid (TEB)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

stain in cellulose acetate membrane

A

ponceaus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q
  • used as a compliment to cellulose acetate electrophoresis
A

Citrate agar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q
  • differentiates some Hb variants that migrate together on cellulose acetate
A

citrate agar hemoglobin electrophoresis

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

negative in CAHE

A

Hb F

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

positive in CAHE

A

Hb C

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

pH of citrate agar electrophoresis

A

6.0-6.2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Hb A becomes Hb S when glutamic acid on the 6th position of the beta chain is replaced by ____

A

valine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

hgb S state that causes sickle cell anemia

A

homozygous state (SS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

hb S state that causes sickle cell trait

A

heterozygous state (AS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

clinical features of sickle cell disease

A
  1. vasooclusive
  2. sequestration
  3. chronic hemolytic anemia
  4. megaloblastic
  5. aplastic
  6. acute chest syndrom
  7. acute infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

recurring pain

A

crises

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

hallmark of sickle cell disease

A

vasooclusive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

gradual loss of splenic function

A

autosplenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

there is a presence of _____ & _____ in autosplenectomy

A

Howell Jolly bodies & Pappenheimer bodies

33
Q
  • shortened RBC survival
A

Chronic hemolytic anemia

34
Q

in chronic hemolytic anemia there is an

  • increased ____ & ____
  • decreased _____ & ____
A
  • retics & jaundice

- Hemoglobn & hematocrit

35
Q

result from sudden arrest of RBC production due to folate depletion

A

Megaloblastic anemia

36
Q
  • most common life threatening hemolytic complication
A

Aplastic anemia (bone marrow failure)

37
Q
  • aplastic anemia is associated with ____
A

parvovirus infection

38
Q

leading cause of death among adults (crises)

A

Acute chest syndrome

39
Q
  • bacterial infections

- most frquent cause of death esp first 3 years of life

A

Acute infections

40
Q

hallmark of sickle cell disease

A

sickle cell & target cell

41
Q

lab findings of sickle cell disease

A

severe normochromic/ normocytic hemolytic anemia w/ polychromasia

42
Q

pbs of sickle cell disease

A

sickle cell, target cells, ovalocytes, schistocytes, RBC, Pappenheimer bodies & Howell Jolly Bodies

43
Q

in scd there is an increased ___ & decreased ___

A

bilirubin & haptoglobin

44
Q

the death of scd is usually because of ____ or ____

A

infection or congestive heart failure

45
Q
  • defect is inherited from one parent
  • one normal beta chain can produce some HbA
  • no clinical symptoms
A

sickle cell trait

46
Q

sickle cell trait has apparent immunity to

A

Plasmodium falciparum

47
Q

occurs in African-American populations

- no Hb A is produced: Approximately

A

Hb C disease (CC)

48
Q
  • rhomboid, tetragonal or rod shaped crystals of dense staining hemoglobin that often destrort the cell.
  • found un patients with CC or SC disease, particularly after splenectomy
A

Hemoglobin C crystals

49
Q

laboratory findings for hemoglobin c

A

normochromic/normocytic anemia w/ target cells

50
Q

____________ are seen in CC disease only

A

typical hexagonal crystal

51
Q

electrophoresis: Hb C migrates with ___, ____, & ___ on ______ electrophoresis

A

HbA2, Hb E & Hb O

alkaline

52
Q

heterozygous form of HbC disease but no symptoms

A

Hb AC

53
Q
  • a double heterozygous condition where S & C are inherited
  • most common compound heterozygous
  • no HbA is produced
A

Hb SC Disease

54
Q

other name of Hb SC

A

Washington monument

55
Q

frequency of Hb SC

A

West africa

56
Q

symptoms of Hb SC

A

less severe than sickle cell anemia but more severe than Hb C disease

57
Q

laboratory findings of Hb SC

A
  • moderate to severe normocytic/normochromic anemia w/ target cells
58
Q
  • protrude from RBC membrane
  • longer than Hb C
  • shorter & thicker than HbS
  • often branched
A

SC crystals

59
Q
  • lysine replaces glutaic acid at position 26 on beta chain

- mild anemia w/ microcytes

A

Hemoglobin E

60
Q

Hb E migrates w/ ____ in ____

A

Hb A2, C & O

alkaline

61
Q

Hb EE resembles _____

A

thalassemia trait

62
Q

Hb E gene has highest incidence in ____

A

Thailand

63
Q
  • malaria prevalent
A

Hb E gene

64
Q

P. falciparum multiplies more slowly in ____

A

Hb EE

65
Q

variants of Hgb D & G

A
  • Hb D-Punjab
  • Hb D-Los Angeles
  • Hb G-Philadelphia
66
Q

homozygous w/ 95% HbD, normal Hb A2 & HbF

A

Hb DD

67
Q

hetero form, asymptomatic

A

Hb D & Hb C

68
Q
  • caused by a variety of mutation in alpha, beta & gamma globin genes
  • substitution of tyrosine amino acid to histidine amino acids
A

Hemoglobin M

69
Q

iron form of Hb M

A

ferric form

70
Q
  • result from genetic mutation to globin gene creating Hb products that precipitate in vivo
A

Unstable Hemoglobin

71
Q

anemias with unstable hemoglobin

A
  • congenital nonspherocytic anemia
  • congenital heinz body anemia
  • unstable hemoglobin disease
72
Q

laboratory test for unstable hemoglobin

A
  • isopropanol precipitation test

- heat denaturation test

73
Q

result for isopropanol precipitation test

A

unstable hemoglobin

74
Q

5 mins result of isopropanol precipitation test

A

rapid ppt

75
Q

after 20 mins result of isopropanol precipitation test

A

heavy flocculation

76
Q

normal hemoglobin result of isopropanol precipitation test

A

more than 40 mins=Hb precipitate

77
Q

result of unstable Hb in heat denaturation test

A

heat sensitive, flocculent ppt

78
Q

result of normal hb in heat denaturation test

A

little or no ppt

Hematology I (11 decks)

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