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Botany > HEMA LEC - Hgbopathy > Flashcards

HEMA LEC - Hgbopathy Flashcards

1
Q

Group of inherited disorders causing structurally abnormal globin chain synthesis due to AA substitutions

A

HEMOGLOBINOPATHIES

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2
Q

HEMOGLOBINOPATHIES are hetero or homozygous disease?

A

• Homozygous disease conditions (both globin chains affected)

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3
Q

what cells are associated with Hgbopathy

A

Target cells and sickle cells

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4
Q

HEMOGLOBINOPATHY DIAGNOSIS [3]

A
  • Hgb electrophoresis
  • Isoelectric focusing
  • DNA (PCR) Analysis
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5
Q

When valine replaces glutamic acid at position 6 on both beta chains

A

Hgb SS

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6
Q

Hgb SS Inherited from one or both parents?

A

• Inherited from both parents

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7
Q

percentage of HbA produced if w/ Hgb SS

A

0%

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8
Q

percentage of HbS produced if w/ Hgb SS

A

80%

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9
Q

percentage of HbF produced if w/ Hgb SS

A

20%

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10
Q

Hb insolubility results when _______ is formed

A

deoxyhemoglobin

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11
Q

Hgb ___ Clinical Findings

• Tissue necrosis

A

Hgb SS

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12
Q

Hgb ___ Clinical Findings

All organs are affected; with kidney failure being a common outcome; hyposplenism and joint swelling also occur

A

Hgb SS

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13
Q

Hgb ___ Clinical Findings
Diagnosis is made after 6 months of age (time of beta-gamma globin chain switch), with life expectancy of 50 years with proper treatment

A

Hgb SS

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14
Q

(time of beta-gamma globin chain switch)

A

6 months of age

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15
Q

Hgb ___ Clinical Findings

Death usually results from infection or congestive heart failure.

A

Hgb SS

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16
Q

Inclusion bodies found in Hb SS [2]

A

Pappenheimer bodies

H-J Bodies

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17
Q

Hgb S migrates with Hemoglobins ? and ? on alkaline hemoglobin electrophoresis; can differentiate using acid electrophoresis

A

D & G

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18
Q

Hgb SS Laboratory Findings Blood picture

bone marrow erythroid hyperplasia (M:E ratio decreases)

A

Severe normocytic/normochromic hemolytic anemia with polychromasia resulting from premature release of reticulocytes

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19
Q

Hgb SS Laboratory Findings

M:E Ratio effect

A

bone marrow erythroid hyperplasia (M:E ratio decreases)

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20
Q

cells present in Hgb SS [3]

A

Sickle cells, target cells, nucRBCs,

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21
Q

Hb __ lab findings

Increased bilirubin and decreased haptoglobin due to hemolysis

A

Hb SS

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22
Q

When valine replaces glutamic acid at position 6 on ONE beta chains

A

Hgb AS

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23
Q

one normal beta chain can produce some Hgb A

A

Hgb AS

24
Q

Percentage of Hgb A in Hgb AS

A

60%

25
Q

Percentage of Hgb S in Hgb AS

A

40%

26
Q

generally produces no clinical symptoms. anemia is rare, but if present, will be normo/normo and sickling can occur during rare crisis states

A

Hgb AS

27
Q

when lysine replaces glutamic acid at position 6 on both beta chains

A

Hgb CC

28
Q

Percentage of Hgb A in Hgb CC

A

0%

29
Q

Percentage of Hgb A2 in Hgb CC

A

2

30
Q

Percentage of Hgb F in Hgb CC

A

7

31
Q

Percentage of Hgb Cin Hgb CC

A

90%

32
Q

Normo/normo anemia with target cells, intracellular rodlike crystals

A

Hgb CC

33
Q

Hb C migrates with Hgb

A

A2, E, O

34
Q

The ___ Hgb C trait patient is asymptomatic, with no anemia, one normal beta chain is able to produce 60% Hb A, 40% Hb C, normal amounts of A2 and F

A

HETEROZYGOUS

35
Q

double heterozygous condition where an abnormal sickle gene from one parent and an abnormal C gene from the other parent is inherited

A

Hgb SC

36
Q

percentage of Hb A in Hgb SC

A

0%

37
Q

percentage of Hb S in Hgb SC

A

50%

38
Q

percentage of Hb C in Hgb SC

A

50%

39
Q

percentage of Hb F in Hgb SC

A

7%

40
Q

moderate to severe normo/normo with target cells, characterized by SC crystals, may see rare sickle cells or C crystals

A

Hgb SC

41
Q

when lysine replaces glutamic acid at position 26 on the beta chain

A

Hgb E

42
Q

homozygous condition results in mild anemia with microcytes and target cells; heterozygotes are asymptomatic

A

Hgb E

43
Q

when glycine replaces glutamic acid at position 121 on the beta chain

A

Hgb D

44
Q

abnormal Hgb variants

HbM: alpha 2 (58th His to Tyr), beta 2

A

Hgb M

45
Q

tetramer of gamma

A

Hb Bart

46
Q

abnormal variant of Hb F

A

Hb Bart

47
Q

Decrease Hb insolubility

A

Hgb SS

48
Q

Immunity to falciparum (2)

A

Hgb SS

Hgb AS

49
Q

+ Hgb Solubility Screening Test

3

A

Hgb SS
Hgb AS
Hgb SC

50
Q

No Hgb A produced (2)

A

Hgb SS

Hgb CC

51
Q

Asymptomatic

A

AS
Heterozygous Hb C
Heterozygous Hb E

52
Q
  1. Occurs most commonly in African-American, African, Mediterranean, and Middle East populations.
A

Sickle Cells Disease (Hgb SS)

53
Q

is the most common hemoglobinopathy in the United States.

A

heterozygous trait

54
Q
  1. Occurs in the African-American And African populations.
A

Hgb C Disease / Hgb CC

55
Q

African, Mediterranean, and Middle Eastern populations

A

Hgb SC Disease

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