HEMOGLOBINOPATHIES Flashcards

memorization

1
Q

No Hb is produced; Hb insolubility results when deoxyhemoglobin is formed. Hb crystallizes, forming the classic sickle shape of the cell

A

Sickle cell disease (Hb SS)

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2
Q

In Sickle cell disease, there is approximately ___% Hb S and ___% Hb F

A

80% Hb S
20% Hb F

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3
Q

Defect is inherited from one parent only; Approximately 60% Hb A and 40% Hb S

A

Sickle cell trait (Hb AS)

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4
Q

No Hb A is produced; approximately 90% Hb C, 2% Hb A2, and 7% Hb F

A

Hb C disease (Hb CC)

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5
Q

Folded cells can be found in what condition?

A

Hb C disease

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6
Q

A double heterozygous condition where an abnormal sickle cell gene from one parent and abnormal C gene from the other parent is inherited; No Hb A is produced

A

Hb SC disease

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7
Q

Hb C crystals can be seen in Hb C disease only and not in Hb SC disease

true or false

A

False

Hb C crystal can be seen in both Hb C and SC disease

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8
Q

Hb SC crystals can be seen in both Hb C and Hb Sc disease

true or false

A

False

Hb SC crystal is exclusively produced in Hb SC disease

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9
Q

Hemoglobin S
____ amino acid of B chain; glutamate is replaced by _____

A

6th amino acid of B chain; glutamate is replaced by Valine

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10
Q

hemoglobin C
____ amino acid of B chain; glutamate is replaced by _____

A

6th amino acid of B chain; glutamate is replaced by Lysine

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11
Q

hemoglobin D
____ amino acid of B chain; glutamate is replaced by _____

A

121st amino acid of B chain; glutamate is replaced by Glysine

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12
Q

hemoglobin E
____ amino acid of B chain; glutamate is replaced by _____

A

26th amino acid of B chain; glutamate is replaced by Lysine

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13
Q

hemoglobin O
____ amino acid of B chain; glutamate is replaced by _____

A

121st amino acid of B chain; glutamate is replaced by Lysine

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14
Q

most anodal hemoglobin in cellulose acetate medium

A

Hb A

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15
Q

most anodal hemoglobin in citrate agar

A

Hb C

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16
Q

Hb pattern in cellulose acetate from most anodal to least anodal

A

(+) A-F-S-C (-)

17
Q

Hb pattern in Citrate agar from most anodal to least anodal

A

(+) C-S-A-F (-)

18
Q

Cellulose acetate medium pH

A

pH 8.4

19
Q

Citrate agar pH

A

pH 6.0-6.2