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Hemoglobinopathies are result due to the differences in the arrangement of what substance?

Amino acids in the polypeptide chains

1

Hb S, C, D, and E are differentiated thru?

Solubility
Mobility in the electrophoresis

2

Charge of hemoglobin molecules

Negative charge

3

Hemoglobin molecules migrate in what pole?

Anode or positive pole

4

In cellulose acetate membrane electrophoresis what Hb is the fastest? Slowest.

Fastest-Hb H and I
Slowest-Hb C, E, O and A2

5

pH of cellulose acetate membrane

8.4-8.6
Alkaline

6

Stain used in cellulose acetate membrane

Ponceau S

7

Used to differentiate Hb S, G and D

Citrate agar membrane

8

These are important agents in the production of hemolytic anemia

Hb S
Hb C
Hb E
Hb D

9

pH of citrate agar

6.2-6.4
Acidic

10

Glutamic acid on the sixth position of the beta chain is replaced by Valine

Hemoglobin S

11

Homozygous state of Hb S causes? Heterozygous causes.

Homo-sickle cell anemia
Hetero-sickle cell trait

12

Differentiates some hemoglobin variants that migrate together on cellulose acetate membrane

Citrate agar membrane

13

RBC become rigid and are trapped in capillaries

Sickle cell anemia

14

Sickle cell anemia is associated with?

Hypersplenism
Joint swelling
Kidney failure

15

Sever normocytic hemolytic anemia with polychromasia

Sickle cell anemia

16

In sickle cell anemia: ____ bilirubin and ____haptoglobin

Increase bilirubin
Decrease haptoglobin

17

In cellulose acetate membrane electrophoresis. Hb S migrates with?

Hb G and D

18

Diagnosis of Hb S is made after?

6 months of age. Time of beta gamma switch

19

Defect is inherited from one parent

Sickle cell trait

20

Sickle cell trait:
Concentration of adult hemoglobin

HbA (60%) + HbS (40%) and normal amount of Hb F and A2

21

Screening test for the detection of sickling hemoglobin

Dithionite tube test

22

In dithionite tube test what reagent used to lysed the red cells

Saponin

23

In dithionite tube test:
This removes oxygen from the test environment resulting to deoxygenated state

Sodium dithionite

24

Buffer used in Cellulose acetate membrane

Tris EDTA boric acid TEB

25

The precipitate formed is consist of this liquid crystal

Tactoids

26

Deoxygenates hemoglobin

Sodium metabisulfite

27

Under deoxygenated state Hb S causes the formation of?

Sickle cells

28

How many drops of sodium metabisulfite if there is normal Hb? How bout decrease Hb?

Normal- 2 drops
Decrease-1 drop

29

This is used to seal the glass slide in sodium metabisulfite test

Petroleum jelly

30

Positive result in sodium metabisulfite test

Holly leaf appearance

31

Concentration of adult hemoglobin in Hb CC disease

HbC (90%) + HbA2 (2%) + HbF (7%)

32

Holly leaf is found in what sickle cell?

Sickle cell trait

33

Glutamic acid on the sixth position is replaced by lysine

Hb C

34

Rhomboid, tetragonal or rod shaped crystals of dense staining hemoglobin that often distort the cell

Hb C crystal

35

Normocytic anemia with target cells

Hb CC

36

In cellulose acetate membrane electrophoresis Hb C migrates with?

Hb E, O, A2

37

Occurs in African-American and African population. With no production of Hb A

Hemoglobin C disease
Hb CC disease

38

Adult hemoglobin concentration in heterozygous Hb C disease

HbA (60%) + HbC (40%)
HbA2 and F normal

39

A double heterozygous condition where S and C are inherited

Hb SC disease

40

Mild anemia with microcytes

Hb E

41

Most common hemoglobinopathy in the US

Sickle cell trait

42

Less severe than sickle cell anemia but more severe that Hb C disease

Hb SC disease

43

Concentration of adult hemoglobin in Hb SC

HbS (50%) + HbC (50%) + HbF (7%)

44

Hb E migrates with?

Hb C, O, A2

45

Glycine replaced glutamic acid in the 121 position in the beta chain

Hb D

46

characterized by a reduction or total absence of synthesis of one or more of the globin chain of hemoglobin

Thalassemia

47

Total lack of beta or alpha chain

Thalassemia major

48

Lysine replaces glutamic acid at position 26th on the beta chain

Hb E

49

Production of alpha or beta at a decrease rate

Thalassemia minor

50

Three gene deletion

Hb H disease

51

Make the solution turbid

Tactoids

52

In the thalassemia syndrome erythrocytes indices indicate a

Microcytic and hypochromic anemia

53

Associated with the decrease production of alpha chains

Alpha thalassemia

54

Hb D migrates with?

Hb S and G

55

Decrease production of beta chain

Beta thalassemia

56

Single cell deletion

Silent carrier state

57

Four gene deletion

Hemoglobin bart's
Hydrops fetalis

58

Composed of four beta chains

Hb H

59

Hb H supravital stain

Brilliant cresyl blue

60

Increase Hb A2 and F

Beta thalassemia

61

Two gene deletion

Alpha thalassemia trait or minor

62

Commonly acquired porphyria that results from a block in several of the heme biosynthetic enzyme

Lead intoxication

63

Thalassemia 's major abnormalities

Basophilic stippling
Target cells

64

Xray result of beta thalassemia

Hairy skull

65

Inherited or acquired disorder of heme sythesis

Porphyria

66

Primary cause of porphyria

Specific enzyme deficiency

67

Produces photosensitivity and skin lesions

Cutaneous porphyria

68

Enzyme defect in heme synthetase

Variegate porphyria

69

Resemble a putted golf ball

Hb H

70

Werewolf

Congenital erythropoietic porphyria

71

Other name of variegate porphyria

Ferrochelatase deficiency

72

Most common neurologic porphyria

Acute intermittent porphyria

73

Lead intoxication enzyme defect

ALA dehydrogenase

74

Most common cutaneous porphyria

Porphyria cutanea tarda

75

Associated with severe photosensitivity

Congenital erythropoietic porphyria

76

Induced by the presence of excess porphyrins in the skin which generates oxygen radicals that attack cells

Cutaneous porphyria

77

Teeth and urine fluoresce under UV light

Congenital erythropoietic porphyria

78

Absence of photosensitivity, and coproporphobilinogen oxidase

Lead intoxication

79

Appear in adult following liver disease or excessive alcohol intake

Porphyria cutanea tarda

80

Typical hexagon crystals are seen in what disease

Hb CC disease

81

This membrane is used only a compliment to cellulose acetate membrane

Citrate agar

82

Toxic granulation in neutrophil

Lead intoxication

83

Qualitative structural defect in hemoglobin

Hemoglobin variants
Hemoglobinopathies

84

Cooley's anemia

B thalassemia major

85

Classical symptoms of anemia

Fatigue
Shortness of breath

86

Concentration of Hb in moderate anemia

7-10g/dL

87

Physiologic adaptation of man to anemia

Primary oxygen sensing system
Heart rate, cardiac output and respiratory rate increased
2,3-BPG increase

88

RBC parameter

RBC count
Hb
Hct

89

RBC mass is normal
Plasma volume is increased

Relative anemia

91

Refers to a decrease in the number of erythroid precursor in the BM resulting in the decrease RBC production and anemia

Insufficient erythropoiesis

93

RBC Indices

MCH
MCV
MCHC

94

Measure of the average RBC volume

MCV

95

Indicates variation in RBC volume within the population measured and correlates with anisocytosis

RBC distribution width

96

Test used to asses the BM ability to increase RBC production in response to anemia

Reticulocyte count

97

Bone marrow exam is performed when

Fever of unknown origin
Suspected hematologic malignancy
Unexplained anemia

98

Transient type of anemia. Wherein secondary to an unrelated condition

Relative anemia

99

RBC mass is decreased
Plasma volume is normal

Absolute anemia

100

Stage of IDA wherein there is a progressive loss of storage iron

Stage 1

101

Lab result in stage 1

Serum iron, TIBC, Hb normal
Serum ferritin decreased

102

Stage 2 is characterized as

Exhaustion of the storage pool of iron

103

Lab results of stage 2

Serum ferritin and serum iron decreased
TIBC increased
FEP increased

104

Frank anemia

Stage 3

105

Lab results of stage 3

Serum iron, ferritin, hemoglobin decreased
TIBC and FEP increased

106

Term for sore tongue

Glossitis

107

Inflamed cracks at the corners of the mouth

Angular chellosis

108

Spooning of the fingernails

Koilonychia

109

Unusual craving

Pica

110

Screening test for anemia

Cbc

111

Diagnostic test for IDA

Serum iron
Serum ferritin
TIBC
Percent saturation

112

Specialized test for IDA

FEP
Iron stain

113

Lab finding for IDA

Microcytic, hypochromic
LOW: serum iron, ferritin, Hb/Hct, RBC indices, reticulocyte count
HIGH: TIBC

114

Anemia due to inability to use available iron for hemoglobin production

Anemia of chronic disease

115

Impaired release of storage iron is associated with increased level of this liver hormone

Hepcidin

116

Lab findings for ACD

Normocytic, normochromic
Increased ESR
Normal to elevated ferritin
Low serum iron and TIBC

117

Second most abundant cause of anemia

ACD

118

Caused by blocks in the protoporphyrin pathway resulting in defective hemoglobin synthesis

Sideroblastic anemia

119

Hallmark of sideroblastic anemia

Ringed sideroblasts

120

Excess iron accumulates in the mitochondrial region of the mature RBC in the peripheral circulation

Siderocytes

121

Excess iron accumulates in the mitochondrial region of the immature RBC in the bone marrow and encircle the nucleus

Ringed sideroblasts

122

Best stain used for sideroblastic anemia

Perl's prussian blue

123

What do you call the inclusions in sideroblastic anemia? Example

Siderotic granule
Pappenheimer bodies

124

A irreversible type of sideroblastic anemia

Primary

125

Reversible type of sideroblastic anemia

Secondary

126

Lab findings fir sideroblastic anemia

Microcytic, hypochromic
Increased ferritin and serum iron
TIBC decreased

127

Multiple blocks in the protoporphyrin pathway

Lead poisoning

128

Lab findings for lead poisoning

Normocytic, normochromic
With coarse basophilic stippling

129

Defect in the DNA synthesis that results in abnormal nuclear maturation

Megaloblastic anemia

130

Exhibits asynchronism

Megaloblastic anemia

131

Laboratory findings for megaloblastic anemia

Pancytopenia
Macrocytic
Normochromic anemia

132

Caused by a deficiency in the intrinsic factor. It has an antibody against IF or parietal cells

Pernicious anemia

133

Symptoms almost the same as B12. No involvement during saturday

Folic acid deficiency

134

Anemia that occurs after the body has a forceful contact with hard surfaces

March hemoglobinuria

135

All extrinsic non immune defects exhibit this lab findings

Normocytic, normochromic

136

Acquired disorders that cause intravascular hemolysis with schistocytes

Extrinsic-nonimmune defect

137

Extrinsic immune defect lab results

Normocytic normochromic

138

All are acquired disorders that cause accelerated destruction with reticulocytosis

Extrinsic-immune defect

139

RBC are coated with IgG or complement causing membrane loss and spherocytes

WAIHA

140

RBC are coated with IgM

CAIHA

141

Antibody present in CAIHA is usually

Anti I

142

Recepient has antibody against the donor cells

Hemolytic transfusion reaction

143

Donath landsteiner test positive

PNH

144

The complement coated with RBC lysed when warmed at what temperature

37C

145

Most common membrane defect

H. Spherocytosis

146

Increase permeability of sodium

H. Spherocytosis

147

Membrane defect caused by polarization of cholesterol

H. Elliptocytosis

148

Abnormal permeability to both sodium and potassium

H. Stomatocytosis

149

Abetalipoproteinemia

H. Acanthocytosis

150

Caused by increased cholesterol:llecithin ration in the membrane due to lipid concentration

H. Acanthocytosis

151

Most common defect in hexose monophosphate shunt

g6PD deficiency

152

Gold standard for iron testing

Iron stains

153

The production of erythroid progenitor cells are defective

Ineffective erythropoiesis