FINALS 1: Hemoglobinopathies Flashcards Preview

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Flashcards in FINALS 1: Hemoglobinopathies Deck (30)
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1

Type of cell that is associated with the hemoglobinopathies

Target cells

2

Disease that is caused when valine replaces glutamic acid at position 6 on both beta chains

Sickle cell disease

3

Sickle cell disease is caused when ______ replaces ______ at position 6 on both beta chains

valine
glutamic acid

4

In sickle cell disease no ____ is produced

Hgb A

5

In sickle cell disease _____ and ____ are seen.

Hgb S and Hgb F

6

Hemoglobin insolubility results when______ is formed.

deoxyhemoglobin

7

Hemoglobin ____ in erythrocytes which causes the sickle shape of the RBC

crystallizes

8

In sickle cell disease, _________ haemolytic anemia with polychromasia resulting from premature release of reticulocytes

normochromic/normocytic

9

In sickle cell disease what happens to the BM

bone marrow erythroid hyperplasia (M:E ratio decreases)

10

Types of cells present in sickle cell disease

Sickle cells
target cells,
nucleated RBCs
Pappenheimer bodies
Howell-Jolly bodies

11

________ is caused when valine replaces glutamic acid at position 6 on one beta chain

Sickle cell trait

12

Number of beta chains affect in sickle cell disease

2

13

This _________ is the most common hemoglobinopathy in the United States

heterozygous trait

14

Sickle cell trait generally produces no clinical symptoms. Anemia is rare but, if present, will be _____________, and sickling can occur during rare crisis states (same as in Hgb SS).

normochromic/normocytic

15

Result of haemoglobin solubility screening test in Sickle cell trait

+

16

Sickle cell trait/disease patients have apparent immunity to what blood parasite

Plasmodium falciparum.

17

Hgb C disease is caused when______ replaces glutamic acid at position 6 on both beta chains.

lysine

18

In Hgb C disease, no _____ is produced

Hgb A

19

In Hgb C disease, ____, ____ and ____ are produced.

Hgb C
Hgb A2
Hgb F

20

In Hgb C disease, ___________ anemia with target cells

normochromic/ normocytic

21

In Hgb C disease is characterized by ________

intracellular rodlike C crystals

22

Method in which it can differentiate hemoglobins

acid electrophoresis

23

The heterozygous Hgb C trait patient is ______, ________ (with/without?) anemia; the_____ normal beta chain is able to produce approximately 60% Hgb A and 40% Hgb C, with normal amounts of Hgb A2 and Hgb F

asymptomatic
without
1

24

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

Hgb SC disease

25

In Hgb SC disease, severe _________ anemia with target cells occur; characterized by ______; may see rare sickle cells or C crystals; positive haemoglobin solubility screening test

normocytic/normochromic
SC crystals

26

In Hemoglobin E, it is caused when______ replaces glutamic acid at position 26 on the beta chain

lysine

27

Homozygous condition of Hemoglobin E results in ______ with microcytes and target cells; heterozygotes are _______

mild anemia
asymptomatic

28

Hemoglobin D also known as ____

Punjab

29

Hemoglobin D is caused when_____ replaces glutamic acid at position 121 on the beta chain

glycine

30

In Hemoglobin D, homozygous is _______and heterozygous conditions are________

symptomatic or asymptomatic

asymptomatic
asymptomatic