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Flashcards in HEMA LEC - Hgbopathy Deck (55)
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1

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

HEMOGLOBINOPATHIES

2

HEMOGLOBINOPATHIES are hetero or homozygous disease?

• Homozygous disease conditions (both globin chains affected)

3

what cells are associated with Hgbopathy

Target cells and sickle cells

4

HEMOGLOBINOPATHY DIAGNOSIS [3]

• Hgb electrophoresis
• Isoelectric focusing
• DNA (PCR) Analysis

5

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

Hgb SS

6

Hgb SS Inherited from one or both parents?

• Inherited from both parents

7

percentage of HbA produced if w/ Hgb SS

0%

8

percentage of HbS produced if w/ Hgb SS

80%

9

percentage of HbF produced if w/ Hgb SS

20%

10

Hb insolubility results when _______ is formed

deoxyhemoglobin

11

Hgb ___ Clinical Findings
• Tissue necrosis

Hgb SS

12

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

Hgb SS

13

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

Hgb SS

14

(time of beta-gamma globin chain switch)

6 months of age

15

Hgb ___ Clinical Findings
Death usually results from infection or congestive heart failure.

Hgb SS

16

Inclusion bodies found in Hb SS [2]

Pappenheimer bodies
H-J Bodies

17

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

D & G

18

Hgb SS Laboratory Findings Blood picture
bone marrow erythroid hyperplasia (M:E ratio decreases)

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

19

Hgb SS Laboratory Findings
M:E Ratio effect

bone marrow erythroid hyperplasia (M:E ratio decreases)

20

cells present in Hgb SS [3]

Sickle cells, target cells, nucRBCs,

21

Hb __ lab findings
Increased bilirubin and decreased haptoglobin due to hemolysis

Hb SS

22

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

Hgb AS

23

one normal beta chain can produce some Hgb A

Hgb AS

24

Percentage of Hgb A in Hgb AS

60%

25

Percentage of Hgb S in Hgb AS

40%

26

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

Hgb AS

27

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

Hgb CC

28

Percentage of Hgb A in Hgb CC

0%

29

Percentage of Hgb A2 in Hgb CC

2

30

Percentage of Hgb F in Hgb CC

7

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