ch 7 normochromic anemias Flashcards
conditions, defects of the rbc, hematology indicators/symptoms (152 cards)
1
Q
this condition has a deficiency in spectrin and ankyrin
A
Hereditary Sphereocytosis
2
Q
increased osmotic fragility; normal cells lyse at 0.45%, this condition causes rbc’s to lyse at 0.65% NaCl
A
hereditary sphereocytosis
3
Q
treatment for hereditary sphereocytosis would be a splenectomy/partial splenectomy. how does this help the patient?
A
reduces blood transfusions and anemia, minimizes gallbladder issues.
4
Q
would these cells have a increased or decreased osmotic fragility? target
cells, sickle cells, iron deficiency anemia, and thalassemia
A
decreased
5
Q
what is the retic count in someone that has H.S?
A
3%-10%
6
Q
symtom in H.S. when your bilirubin is elevated
A
jaundice
7
Q
symtom in H.S where your h&h and rbc are low
A
anemia
8
Q
symptom in H.S. when your spleen is enlarged
A
splenomegaly
9
Q
rdw slightly elevated in H.S. which may indicate what kind of indices
A
anisocytosis or poikilocytosis
10
Q
Cholelithiasis (condition characterized by the presence of gallstones in the gallbladder) what anemic condition is associated with this
A
hereditary sphereocytosis (H.S.)
11
Q
marked polychromasia and nrbc’s seen in what hereditary condition
A
hereditary sphereocytosis
12
Q
this organ inspects cells for inclusions, parasites abnormal hgb products, and abnormal membrane and acts as a filter
A
spleen
13
Q
RBC’s are less or more elastic in sphereocytes
A
less
14
Q
patients with this condition show moderate anemia and have elevated (MCHC) >, (MCV) is low normal and (RDW) is slightly elevated
A
HS hereditary sphereocytosis
15
Q
increased mchc combined with elevated rdw is a strong predictive value in screening for what
A
HS hereditary sphereocytosis
16
Q
primary dx lab test for HS (hereditary sphereocytosis)?
A
osmatic fragility test
17
Q
this medication is offered after splenectomy in younger children
A
prophylactic penicillin
18
Q
the condition that causes deficiency in spektrin and the proteins commonly associated with the alpha and beta spektrin regions.
A
Hereditary Elliptocytosis
19
Q
two clinical variants considered under common HE, range from silent carrier state to what state
A
transfusion dependent
20
Q
Southeast Asian Ovalocytosis is a subtype of what disease
A
Hereditary Elliptocytosis
21
Q
Red cells are spoon shaped;
appear to have two bars
across the center
A
Southeast Asian Ovalocytosis
22
Q
Cells strongly resistant to
heat and are rigid, Provides mild protection
against malaria
A
Southeast Asian Ovalocytosis
23
Q
which BAND has a well-defined molecular defect in Southeast Asian Ovalocytosis
A
Band 3
24
Q
hemolysis may or may not be present in this disorder
A
Southeast Asian Ovalocytosis
25
RBC's in this disorder are strongly heat RESISTANT and rigid, they are able to maintain their shape under temperatures that cause normal rbc's to crenate or burst.
Southeast Asian Ovalocytosis
26
a cross between HE and HS
Spherocytic Hereditary
Elliptocytosis
27
northern European ancestry, Spherocytes and elliptocytes present, Mild hemolysis, Increased osmotic fragility, and gallbladder disease
Spherocytic Hereditary Elliptocytosis
28
Rare recessive disorder in African
Americans
Hereditary
Pyropoikilocytosis
29
Cells crenate and hemolyze at 46 C
* Normal cells fragment at 49 C
Hereditary
Pyropoikilocytosis
30
Severe hemolysis, poor growth, and facial
deformities
* Red cell budding, rare elliptocytes, and
spherocytes
* Hemoglobin: less than 6.5 g/dL
Low MCV 50 to 75 fL
Hereditary
Pyropoikilocytosis
31
Rare hemolytic disorder
* Intrinsic defect in Na and K permeability
↓ MCHC, ↑ MCV
Hereditary Stomatocytosis
32
Autosomal dominant
Deficiency in stomatin
Hereditary Stomatocytosis
33
Na increases → increase
water → RBC swells
Hereditary Stomatocytosis
34
R B C have slits or bars in
center
* “Smiling”
Stomacytes
35
May also be seen in Rh null
disease
Stomacytes
36
RBC dehydrated, irreversible K+ loss→ hemoglobin puddles at one end
Hereditary Xerocytosis
37
Rare autosomal dominant
RBC ↑ surface:volume ratio
Moderate to severe anemia
Decreased osmotic fragility
Hereditary Xerocytosis
38
Essential enzyme in the pentose phosphate
pathway
G6PD
39
If G6PD is not available rbc is vulnerable to denaturation and what
intravascular hemolysis
40
X-linked recessive disorder
Protection during malarial infections
G6PD
41
this condition is an enzyme deficiency in the EMP that is unable to reduce glutathione to protect rbc's; it is also a sex linked disorder
G6PD
42
drug induced acute hemolytic anemia due to G6PD may cause h&h to go down within how many hours?
24-48
43
RBC'S - Crenated, fragmented, budding
hereditary pyropoikilocytosis
44
increase sensitivity to heat; hemolysis at 46 C
hereditary pyropoikilocytosis
45
rbc's hemolyze at what temp in hereditary pyropoikilocytosis?
46 C
46
antibody directed against antigen
T cells suppress stem cell proliferation
Aplastic anemia
47
caused by viruses, chemicals, or radiation
aplastic anemia
48
main symptom of aplastic anemia would be
pancytopenia (decreased RBC, WBC, PLT)
49
hypoproliferative disorder (pancytopenia)
aplastic anemia
50
factors that seem to predispose an individual to an aplastic episode
radiation, chemotherapy/chemicals, benzene, viruses (EBV, HBV, HCV)
51
treatment for aplastic anemia
transfusion, steroid, or stem cell transplant
52
in this condition the RBC membrane lacks the protein PIGA
Paroxysmal nocturnal hemoglobinuria
53
RBC's are susceptible to complement lysis due to somatic mutation
Paroxysmal nocturnal hemoglobinuria
54
what is the primary manifestation of Paroxysmal nocturnal hemoglobinuria
intravascular lysis
55
Paroxysmal nocturnal hemoglobinuria treatment
Transfusion, bone marrow transplant, iron therapy, drug eculizumab
56
what protein does paroxysmal nocturnal hemoglobinuria lack?
PIGA
57
What CD cells are affected by somatic mutation in Paroxysmal nocturnal hemoglobinuria
CD 55 and CD 59
58
hemolytic anemia, rbc's destroyed at night
paroxysmal nocturnal hemoglobinuria
59
what is present in the morning with someone who has PNH?
hemoglobinuria
60
what's is a PBS of someone who has PNH?
Heinz bodies
61
what test are used for paroxysmal nocturnal hemoglobinuria (PNH)?
sucrose hemolysis test (sugar water)
acid hemolysis test (hams test)
flow cytometry
FLAER
62
clinical lab symptoms of PNH ?
pancytopenia; increased relic count
63
Anti-P attaches to rbc's at low tempetures
paroxysmal cold hemoglobinuria
64
rare hemolytic anemia caused by anti p
paroxysmal cold hemoglobinuria
65
activates compliment at warmer temps; attaches to the rbc's at lower temp
paroxysmal cold hemoglobinuria
66
treatment for paroxysmal cold hemoglobinuria
transfusions
67
more jaundice than anemia
neonatal jaundice
68
increased sensitivity to vitamin k substitutes, triple dye, and camphorated powder
neonatal jaundice
69
rbc's activate complement at warm temps
paroxysmal cold hemoglobinuria
70
caused by IgM autoantibody of wide thermal range
cold agglutinin syndrome
71
may be caused by mycoplasma pneumonia
cold agglutinin syndrome
72
in Cold Agglutinin Syndrome, Complement is fixed on the red cells during what degrees?
0-5C
73
in cold agglutinin syndrome red cells agglutinate and hemolyze at a body temp of what?
20-25 C
74
treatment for cold agglutinin syndrome
move to a warmer environment
75
the screening test for paroxysmal cold hemoglobinuria
Donath-Landsteiner test
76
what is the defect is the cold agglutinin syndrome?
IgM autoantibody
77
cold agglutinin syndrome binds complement at what temp?
0-5 C
78
at what temp does lysis occur in cold agglutinin syndrome?
25-30C
79
at what temp should bcc samples be incubated and retested for cold agglutinin syndromes ?
37C
80
what disorder has a positive DAT test?
cold agglutinin syndrome
81
which bacteria could cause cold agglutinin syndrome?
mycoplasma pneumonia
82
symptoms: acrocyanosis (numbness bluish tone of fingers and toes) pallor, fatigue, weightless. what condition?
cold agglutinin syndrome
83
what disorder does a abc need to be corrected for falsely increased RBC and HCT?
cold agglutinin syndrome
84
RBC's with hemoglobin puddling on cell's periphery?
hereditary xerocytosis
85
which disorder has valine substituted for glutamic acid in the 6th position?
sickle cell anemia (Hgb SS)
86
which disorder has lysine substituted for glutamic acid in the 6th position?
hemoglobin C (Hgb CC)
87
Beta chain variant located on chromosome 11?
sickle cell anemia (Hgb SS)
88
homozygous inheritance
Hgb S
89
prominent in African Americans
sickle cell anemia
90
red cells containing hemoglobin s are rigid in areas of oxygen concentration
sickle cell anemia
91
forms tactoids that appear as long thin bundles of fiber under electron microscopy
Hgb S
92
form irreversible sickled cells that cause obstruction in blood vessels increasing viscosity of blood and slowing of circulation
sickle cell anemia
93
usually diagnosed through neonatal screening. rbc's are protected from sickling due to high hgb F. this would cause a false positive
sickle cell anemia
94
symptoms of sickle cell anemia
chronic hemolytic process; hyper cellular bone marrow
95
RBC's life span 10-20 days
sickle cell anemia
96
laboratory dx of sickle cell anemia
normocytic/normochromic
decreased Hgb 6-8; Hct, RBC
increased retic, LDH, bilirubin
mcv-slightly elevated
97
these patients live normal lives and have less rbc abnormalities
sickle cell trait (Hgb S)
98
mild chronic anemia
moderate splenomegaly
Hgb C disease (Hgb CC)
99
crystals in PBS that look like bars of gold, which inclusions are these?
Hgb C
100
lab dx of Hgb C
increased MCHC; Hgb 9-12;
increased target cells; NRBC's; schistocytes; moderate reticulocytosis
101
inherited; asymptomatic hemoglobin trait
Hgb C trait
102
Hgb SC disease lab dx
Hgb 8-10; target cells; NRBC's; polychromasia; sickle cells; SC crystals "Washington Monument"
103
list conditions that may precipitate a sickle cell crisis
fever, dehydration, cold, stress
104
sickle cell anemia (Hgb SS)
Cellulose acetate at pH 8.6
Citrate agar at pH 6.2
Hgb S migrates with D,G, Lepore
Hgb S separates from others
105
Hgb C Disease (Hgb CC)
Cellulose acetate at pH 8.6
Citrate agar at pH 6.2
Hgb C migrates with A2, E, OArab, CHarlem
Separates C from others
106
Hgb EE
Cellulose acetate at pH 8.6
Citrate agar at pH 6.2
Hgb E migrates with A2, C, OArab, CHarlem
107
database of information about sickle cell patients and their complications
cooperative study of sickle cell disease
108
treatment used to reduce crisis
Prophylactic antibotics
-pneumoccal vaccines
-Transfusions 3-6 weeks
-Hydroxyurea
109
Cellulose acetate ph 8.6 cathode (-) to anode +) order:
C,S,F,A
110
Citrate agar ph 6.2 cathode (-) to anode (+) order
F,A,S,C
111
Which hgb combination is used for the positive sickle screen control?
AS
112
Which hgb combination is used for the negative sickle screen control?
AA
113
If the solution is clear and the lines behind the tube are visible, the inteperation is neg or positive ?
Negative
114
If the solution is turbid and the lines behind the tube are invisible, the interpretation is neg or positive?
Positive
115
What should the tech do if the patients hemoglobin is less that 8g/ dl?
Double the sample
116
Why shouldn't the sickle cell screening test be performed on infants younger than 6 months?.
due to the presence of Hgb F giving a false positive
117
If the sickle screen test is positive, does the patient have sickle cell disease?
no other variants could be involved
118
Sickle Cell Trait - heterozygous
AS
119
Clinical and Laboratory Features of Sickle cell anemia (Hgb SS):
positive solubility test; Hgb S present on electrophoresis
120
Clinical and laboratory features of Sickle cell trait (Hgb AS):
Hgb S on electrophoresis
normal CBC on PBS
121
Clinical and laboratory features of Hemoglobin C (Hgb CC):
Hgb C crystals
122
Hgb SC crystals
Hgb SC disease
123
microcytic hypochromic anemia
Hgb SC disease
124
Alpha chain variant
African americans
Hgb G Philia
125
Alkaline electrophoresis shows a single, slow-moving band at same position as A2
Hgb C disease
126
Hemoglobin A-C trait on alkaline electrophoresis
Hgb C trait
127
50% hemoglobin S and 50% hemoglobin C on alkaline electrophoresis
Hgb SC Disease
128
Two bands present on electrophoresis: one at hemoglobin S (50%) and one at hemoglobin A2
S Beta Thalassemia
129
band at same position as Hgb C or
A2 on alkaline electrophoresis
Hemoglobin E
130
Migrates to same location as hemoglobin C and must be distinguished
Hgb OArab
131
Citrate electrophoresis at p H 6.4 will isolate this band for positive identification
Hgb OArab
132
Which of the following inclusions cannot be visualized by the Wright-stained peripheral smear?
a. Basophilic stippling
b. Hgb H inclusion bodies
c. Howell-Jolly bodies
d. Heinz bodies
D. Heinz bodies
133
Which of the following functions most
affect spherocytes as they travel through the circulation?
a. They tend to form inclusion bodies.
b. They are less deformable and more sensitive to the low glucose in the spleen.
c. They tend to be sequestered in the spleen because of abnormal hemoglobin.
d. They form siderotic granules and cannot navigate the circulation.
B. They are less deformable and more sensitive to the low glucose in the spleen.
134
Many individuals with hereditary spherocytosis are prone to jaundice because of:
a. Epstein-Barr virus.
b. Pathologic fractures.
c. Gallstone disease.
d. Skin pigmentation.
C. Gallstone disease
135
Which of the following are characteristics of hereditary pyropoikilocytosis?
a. Elliptocytes with spherocytes intermixed in the peripheral smear
b. Spherocytes with polychromasia and low MCV
c. Elliptocytes, spherocytes, and budding red blood cells
d. Mostly elliptocytes with a few other
morphologies
C. Elliptocytes, spherocytes, and budding red blood cells
136
Which red blood cell morphology is formed as a result of Heinz bodies being pitted from the red blood cell?
a. Acanthocytes
b. Bite cells
c. Burr cells
d. Stomatocytes
B. Bite cells
137
Which of the following hemolytic disorders has red blood cells that are especially sensitive to lysis by complement?
a. Paroxysmal nocturnal hemoglobinuria
b. Fanconi's anemia
c. Aplastic anemia
d. Hereditary spherocytosis
A. Paroxysmal nocturnal hemoglobinuria
138
All the following statements are correct about G6PD deficiency except:
a. It is the most common glycolytic enzyme deficiency associated with the pentose phosphate pathway.
b. It increases formation of Heinz bodies.
c. It causes hemoglobin to be oxidized from the ferrous to the ferric state.
d. It is transmitted by a mutant gene located on the X chromosome.
C. It causes hemoglobin to be oxidized from the ferrous to the ferric state.
139
The most common G6PD
genotype that is disease causing 1s
a. Gd Canton.
b. Gd B+.
c. GdMed
d. Gd A-.
C. GdMed
140
An anemia that manifests as decreased marrow cellularity, cytopenias in two cellular elements, and a reticulocytopenia is appropriately termed:
a. Megaloblastic anemia.
b. Aplastic anemia.
c. Sideroblastic anemia.
d. Iron deficiency anemia.
B. Aplastic anemia
141
Individuals with Fanconis anemia characteristically show
a. Intravascular hemolysis.
b. Increased Hgb E
c. Ringed sideroblasts.
d. Thrombocytosis.
B. Increased Hgb E
142
What is the amino acid substitution in patients with sickle cell anemia?
a. Adenine for thymine
b. Lysine for valine
c. Valine for glutamic acid
d. Cytosine for guanine
C. Valine for Glutamic acid
143
Which of the following factors contributes to the pathophysiology of sickling?
a. Increased iron concentration
b. Hypochromia
c. Fava beans
d. Dehydration
D. Dehydration
144
Which of the following statements pertains to most clinically significant hemoglobin variants?
a. Most are fusion hemoglobins.
b. Most are single amino acid substitutions.
c. Most are synthetic defects.
d. Most are extensions of the amino acid chain.
B. Most are single amino acid substitutions.
145
Which of the following hemoglobins ranks second in variant hemoglobins worldwide?
a. Hgb S
b. Hgb E
c. Hgb H
d. Hgb C
B. Hgb E
146
Which of the following shows
crystals appearing like bars of
gold in the peripheral smear?
a. Hgb CC disease
b. Hgb DD disease
c. Hgb EE disease
d. Hgb SS disease
A. Hgb CC disease
147
Which of the following conditions is the leading cause of hospitalization for sickle cell patients?
a. Acute chest syndrome
b. Priapism
c. Painful crisis
d. Splenic sequestration
A. Acute chest syndrome
148
Which of the following hemoglobin separation methods is used for most newbom hemoglobin screening?
a. High-performance liquid chromatography
b. Alkaline electrophoresis
c. Isoelectric focusing
d. Acid electrophoresis
C. Isoelectric focusing
149
Which hemoglobins migrate to the same location at pH 8.6?
a. Hgb F, Hgb H, Hgb Barts
b. Hgb A2, Hgb S, Hgb C
c. Hgb A2, Hgb C, Hgb E
d. Hgb D, Hgb O, Hgb F
C. Hgb A2, Hgb C, Hgb E
150
When nucleated red blood cells are present, a correction is necessary on total WBC because;
a. Nucleated red blood cells are counted as leukocytes
b. The leukocyte count should be higher.
c. The erythrocyte count is too loW.
d. Nucleated red blood cells are confused with platelets.
A. Nucleated red blood cells are counted as leukocytes
151
All but one of the following may be seen in the peripheral smear when a sickle crisis has occurred.
a. Target cells
b. Nucleated red blood cells
c. Polychromasia
d. Moderate elliptocytes
D. Moderate elliptocytes
152
