Anemias Flashcards
0
Q
Hb S, C, D, and E are differentiated thru?
A
Solubility
Mobility in the electrophoresis
1
Q
Hemoglobinopathies are result due to the differences in the arrangement of what substance?
A
Amino acids in the polypeptide chains
2
Q
Charge of hemoglobin molecules
A
Negative charge
3
Q
Hemoglobin molecules migrate in what pole?
A
Anode or positive pole
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
5
Q
pH of cellulose acetate membrane
A
8.4-8.6
Alkaline
6
Q
Stain used in cellulose acetate membrane
A
Ponceau S
7
Q
Used to differentiate Hb S, G and D
A
Citrate agar membrane
8
Q
These are important agents in the production of hemolytic anemia
A
Hb S
Hb C
Hb E
Hb D
9
Q
pH of citrate agar
A
6.2-6.4
Acidic
10
Q
Glutamic acid on the sixth position of the beta chain is replaced by Valine
A
Hemoglobin S
11
Q
Homozygous state of Hb S causes? Heterozygous causes.
A
Homo-sickle cell anemia
Hetero-sickle cell trait
12
Q
Differentiates some hemoglobin variants that migrate together on cellulose acetate membrane
A
Citrate agar membrane
13
Q
RBC become rigid and are trapped in capillaries
A
Sickle cell anemia
14
Q
Sickle cell anemia is associated with?
A
Hypersplenism
Joint swelling
Kidney failure
15
Q
Sever normocytic hemolytic anemia with polychromasia
A
Sickle cell anemia
16
Q
In sickle cell anemia: ____ bilirubin and ____haptoglobin
A
Increase bilirubin
Decrease haptoglobin
17
Q
In cellulose acetate membrane electrophoresis. Hb S migrates with?
A
Hb G and D
18
Q
Diagnosis of Hb S is made after?
A
6 months of age. Time of beta gamma switch
19
Q
Defect is inherited from one parent
A
Sickle cell trait
20
Q
Sickle cell trait:
Concentration of adult hemoglobin
A
HbA (60%) + HbS (40%) and normal amount of Hb F and A2
21
Q
Screening test for the detection of sickling hemoglobin
A
Dithionite tube test
22
Q
In dithionite tube test what reagent used to lysed the red cells
A
Saponin
23
Q
In dithionite tube test:
This removes oxygen from the test environment resulting to deoxygenated state
A
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
