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Hematology > Anemias > Flashcards

Anemias Flashcards

0
Q

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

A

Solubility

Mobility in the electrophoresis

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1
Q

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

A

Amino acids in the polypeptide chains

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2
Q

Charge of hemoglobin molecules

A

Negative charge

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3
Q

Hemoglobin molecules migrate in what pole?

A

Anode or positive pole

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4
Q

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

A

Fastest-Hb H and I

Slowest-Hb C, E, O and A2

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5
Q

pH of cellulose acetate membrane

A

8.4-8.6

Alkaline

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6
Q

Stain used in cellulose acetate membrane

A

Ponceau S

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7
Q

Used to differentiate Hb S, G and D

A

Citrate agar membrane

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8
Q

These are important agents in the production of hemolytic anemia

A

Hb S
Hb C
Hb E
Hb D

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9
Q

pH of citrate agar

A

6.2-6.4

Acidic

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10
Q

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

A

Hemoglobin S

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11
Q

Homozygous state of Hb S causes? Heterozygous causes.

A

Homo-sickle cell anemia

Hetero-sickle cell trait

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12
Q

Differentiates some hemoglobin variants that migrate together on cellulose acetate membrane

A

Citrate agar membrane

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13
Q

RBC become rigid and are trapped in capillaries

A

Sickle cell anemia

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14
Q

Sickle cell anemia is associated with?

A

Hypersplenism
Joint swelling
Kidney failure

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15
Q

Sever normocytic hemolytic anemia with polychromasia

A

Sickle cell anemia

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16
Q

In sickle cell anemia: ____ bilirubin and ____haptoglobin

A

Increase bilirubin

Decrease haptoglobin

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17
Q

In cellulose acetate membrane electrophoresis. Hb S migrates with?

A

Hb G and D

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18
Q

Diagnosis of Hb S is made after?

A

6 months of age. Time of beta gamma switch

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19
Q

Defect is inherited from one parent

A

Sickle cell trait

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20
Q

Sickle cell trait:

Concentration of adult hemoglobin

A

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

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21
Q

Screening test for the detection of sickling hemoglobin

A

Dithionite tube test

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22
Q

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

A

Saponin

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23
Q

In dithionite tube test:

This removes oxygen from the test environment resulting to deoxygenated state

A

Sodium dithionite

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24
Q

Buffer used in Cellulose acetate membrane

A

Tris EDTA boric acid TEB

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25
Q

The precipitate formed is consist of this liquid crystal

A

Tactoids

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26
Q

Deoxygenates hemoglobin

A

Sodium metabisulfite

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27
Q

Under deoxygenated state Hb S causes the formation of?

A

Sickle cells

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28
Q

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

A

Normal- 2 drops

Decrease-1 drop

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29
Q

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

A

Petroleum jelly

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30
Q

Positive result in sodium metabisulfite test

A

Holly leaf appearance

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31
Q

Concentration of adult hemoglobin in Hb CC disease

A

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

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32
Q

Holly leaf is found in what sickle cell?

A

Sickle cell trait

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33
Q

Glutamic acid on the sixth position is replaced by lysine

A

Hb C

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34
Q

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

A

Hb C crystal

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35
Q

Normocytic anemia with target cells

A

Hb CC

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36
Q

In cellulose acetate membrane electrophoresis Hb C migrates with?

A

Hb E, O, A2

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37
Q

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

A

Hemoglobin C disease

Hb CC disease

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38
Q

Adult hemoglobin concentration in heterozygous Hb C disease

A

HbA (60%) + HbC (40%)

HbA2 and F normal

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39
Q

A double heterozygous condition where S and C are inherited

A

Hb SC disease

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40
Q

Mild anemia with microcytes

A

Hb E

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41
Q

Most common hemoglobinopathy in the US

A

Sickle cell trait

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42
Q

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

A

Hb SC disease

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43
Q

Concentration of adult hemoglobin in Hb SC

A

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

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44
Q

Hb E migrates with?

A

Hb C, O, A2

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45
Q

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

A

Hb D

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46
Q

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

A

Thalassemia

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47
Q

Total lack of beta or alpha chain

A

Thalassemia major

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48
Q

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

A

Hb E

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49
Q

Production of alpha or beta at a decrease rate

A

Thalassemia minor

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50
Q

Three gene deletion

A

Hb H disease

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51
Q

Make the solution turbid

A

Tactoids

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52
Q

In the thalassemia syndrome erythrocytes indices indicate a

A

Microcytic and hypochromic anemia

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53
Q

Associated with the decrease production of alpha chains

A

Alpha thalassemia

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54
Q

Hb D migrates with?

A

Hb S and G

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55
Q

Decrease production of beta chain

A

Beta thalassemia

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56
Q

Single cell deletion

A

Silent carrier state

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57
Q

Four gene deletion

A

Hemoglobin bart’s

Hydrops fetalis

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58
Q

Composed of four beta chains

A

Hb H

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59
Q

Hb H supravital stain

A

Brilliant cresyl blue

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60
Q

Increase Hb A2 and F

A

Beta thalassemia

61
Q

Two gene deletion

A

Alpha thalassemia trait or minor

62
Q

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

A

Lead intoxication

63
Q

Thalassemia ‘s major abnormalities

A

Basophilic stippling

Target cells

64
Q

Xray result of beta thalassemia

A

Hairy skull

65
Q

Inherited or acquired disorder of heme sythesis

A

Porphyria

66
Q

Primary cause of porphyria

A

Specific enzyme deficiency

67
Q

Produces photosensitivity and skin lesions

A

Cutaneous porphyria

68
Q

Enzyme defect in heme synthetase

A

Variegate porphyria

69
Q

Resemble a putted golf ball

A

Hb H

70
Q

Werewolf

A

Congenital erythropoietic porphyria

71
Q

Other name of variegate porphyria

A

Ferrochelatase deficiency

72
Q

Most common neurologic porphyria

A

Acute intermittent porphyria

73
Q

Lead intoxication enzyme defect

A

ALA dehydrogenase

74
Q

Most common cutaneous porphyria

A

Porphyria cutanea tarda

75
Q

Associated with severe photosensitivity

A

Congenital erythropoietic porphyria

76
Q

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

A

Cutaneous porphyria

77
Q

Teeth and urine fluoresce under UV light

A

Congenital erythropoietic porphyria

78
Q

Absence of photosensitivity, and coproporphobilinogen oxidase

A

Lead intoxication

79
Q

Appear in adult following liver disease or excessive alcohol intake

A

Porphyria cutanea tarda

80
Q

Typical hexagon crystals are seen in what disease

A

Hb CC disease

81
Q

This membrane is used only a compliment to cellulose acetate membrane

A

Citrate agar

82
Q

Toxic granulation in neutrophil

A

Lead intoxication

83
Q

Qualitative structural defect in hemoglobin

A

Hemoglobin variants

Hemoglobinopathies

84
Q

Cooley’s anemia

A

B thalassemia major

85
Q

Classical symptoms of anemia

A

Fatigue

Shortness of breath

86
Q

Concentration of Hb in moderate anemia

A

7-10g/dL

87
Q

Physiologic adaptation of man to anemia

A

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

88
Q

RBC parameter

A

RBC count
Hb
Hct

89
Q

RBC mass is normal

Plasma volume is increased

A

Relative anemia

91
Q

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

A

Insufficient erythropoiesis

93
Q

RBC Indices

A

MCH
MCV
MCHC

94
Q

Measure of the average RBC volume

A

MCV

95
Q

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

A

RBC distribution width

96
Q

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

A

Reticulocyte count

97
Q

Bone marrow exam is performed when

A

Fever of unknown origin
Suspected hematologic malignancy
Unexplained anemia

98
Q

Transient type of anemia. Wherein secondary to an unrelated condition

A

Relative anemia

99
Q

RBC mass is decreased

Plasma volume is normal

A

Absolute anemia

100
Q

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

A

Stage 1

101
Q

Lab result in stage 1

A

Serum iron, TIBC, Hb normal

Serum ferritin decreased

102
Q

Stage 2 is characterized as

A

Exhaustion of the storage pool of iron

103
Q

Lab results of stage 2

A

Serum ferritin and serum iron decreased
TIBC increased
FEP increased

104
Q

Frank anemia

A

Stage 3

105
Q

Lab results of stage 3

A

Serum iron, ferritin, hemoglobin decreased

TIBC and FEP increased

106
Q

Term for sore tongue

A

Glossitis

107
Q

Inflamed cracks at the corners of the mouth

A

Angular chellosis

108
Q

Spooning of the fingernails

A

Koilonychia

109
Q

Unusual craving

A

Pica

110
Q

Screening test for anemia

A

Cbc

111
Q

Diagnostic test for IDA

A

Serum iron
Serum ferritin
TIBC
Percent saturation

112
Q

Specialized test for IDA

A

FEP

Iron stain

113
Q

Lab finding for IDA

A

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

114
Q

Anemia due to inability to use available iron for hemoglobin production

A

Anemia of chronic disease

115
Q

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

A

Hepcidin

116
Q

Lab findings for ACD

A

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

117
Q

Second most abundant cause of anemia

A

ACD

118
Q

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

A

Sideroblastic anemia

119
Q

Hallmark of sideroblastic anemia

A

Ringed sideroblasts

120
Q

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

A

Siderocytes

121
Q

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

A

Ringed sideroblasts

122
Q

Best stain used for sideroblastic anemia

A

Perl’s prussian blue

123
Q

What do you call the inclusions in sideroblastic anemia? Example

A

Siderotic granule

Pappenheimer bodies

124
Q

A irreversible type of sideroblastic anemia

A

Primary

125
Q

Reversible type of sideroblastic anemia

A

Secondary

126
Q

Lab findings fir sideroblastic anemia

A

Microcytic, hypochromic
Increased ferritin and serum iron
TIBC decreased

127
Q

Multiple blocks in the protoporphyrin pathway

A

Lead poisoning

128
Q

Lab findings for lead poisoning

A

Normocytic, normochromic

With coarse basophilic stippling

129
Q

Defect in the DNA synthesis that results in abnormal nuclear maturation

A

Megaloblastic anemia

130
Q

Exhibits asynchronism

A

Megaloblastic anemia

131
Q

Laboratory findings for megaloblastic anemia

A

Pancytopenia
Macrocytic
Normochromic anemia

132
Q

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

A

Pernicious anemia

133
Q

Symptoms almost the same as B12. No involvement during saturday

A

Folic acid deficiency

134
Q

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

A

March hemoglobinuria

135
Q

All extrinsic non immune defects exhibit this lab findings

A

Normocytic, normochromic

136
Q

Acquired disorders that cause intravascular hemolysis with schistocytes

A

Extrinsic-nonimmune defect

137
Q

Extrinsic immune defect lab results

A

Normocytic normochromic

138
Q

All are acquired disorders that cause accelerated destruction with reticulocytosis

A

Extrinsic-immune defect

139
Q

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

A

WAIHA

140
Q

RBC are coated with IgM

A

CAIHA

141
Q

Antibody present in CAIHA is usually

A

Anti I

142
Q

Recepient has antibody against the donor cells

A

Hemolytic transfusion reaction

143
Q

Donath landsteiner test positive

A

PNH

144
Q

The complement coated with RBC lysed when warmed at what temperature

A

37C

145
Q

Most common membrane defect

A

H. Spherocytosis

146
Q

Increase permeability of sodium

A

H. Spherocytosis

147
Q

Membrane defect caused by polarization of cholesterol

A

H. Elliptocytosis

148
Q

Abnormal permeability to both sodium and potassium

A

H. Stomatocytosis

149
Q

Abetalipoproteinemia

A

H. Acanthocytosis

150
Q

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

A

H. Acanthocytosis

151
Q

Most common defect in hexose monophosphate shunt

A

g6PD deficiency

152
Q

Gold standard for iron testing

A

Iron stains

153
Q

The production of erythroid progenitor cells are defective

A

Ineffective erythropoiesis

Hematology (7 decks)

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