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IBI Test 3 > B3-035 CBCL Hemolytic Anemia > Flashcards

B3-035 CBCL Hemolytic Anemia Flashcards

1
Q

defect in RBC itself

A

intrinsic

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2
Q

defect/problem outside RBC, but affects RBC

A

extrinsic

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3
Q

RBC breakdown with massive release of free hemoglobin in circulation

A

intravascular

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4
Q

RBC breakdown in reticuloendothelial system cells with capture of hemoglobin

A

extravascular

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5
Q

extravascular hemolysis is due to

A

decreased flexibility of the RBC

**minor leak of hemoglobin still occurs

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6
Q

in extravascular hemolysis, serum free hemoglobin is

A

absent

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7
Q

in intravascular hemolysis, serum free hemoglobin is

A

present

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8
Q

in extravascular hemolysis, urine hemoglobin is

A

absent

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9
Q

in intravascular hemolysis, urine hemoglobin is

A

present

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10
Q

hypochromic, microcytic cells are associated with

A

thalassemia

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11
Q

normocytic, normochromic cells are associated with

A

anemia

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12
Q

shistocytes are associated with

A

microangiopathic hemolytic anemia

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13
Q

spherocytes are associated with

A

hereditary spherocytosis
warm autoimmune hemolytic anemia

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14
Q

RBC agglutination is associated with

A

cold autoimmune hemolytic anemia

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15
Q
A

sickle cell

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16
Q
A

sperocytes

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17
Q
A

shistocytes

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18
Q
A

RBC agglutination

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19
Q

structurally abnormal hemoglobin due to mutation in alpha or beta globin gene

A

hemoglobinopathy

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20
Q

hemoglobinopathy is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

extravascular, intrinsic, inherited

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21
Q

thalassemia is
[intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

extravascular, intrinsic, inherited

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22
Q

decreased production of alpha or beta chains due to mutations

A

thalassemia

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23
Q

membrane defect in cytoskeletal protein

A

hereditary spherocytosis

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24
Q

hereditary spherocytosis is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

extravascular, intrinsic, inherited [AD]

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25
Q

spherocytosis is due to

A

increased membrane rigidity with membrane loss

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26
Q

proteins affected by hereditary spherocytosis

A

ankyrin
spectrin
band 3
band 4.2

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27
Q

caused by decreased level of critical enzyme

A

G6PD deficiency

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28
Q

G6PD deficiency causes increased sensitivity to

A

oxidative stress

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29
Q

G6PD deficiency can be precipitated by

A

infection
drugs (primaquine)
foods (fava beans)

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30
Q

G6PD deficiency offers protection from

A

malaria

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31
Q

G6PD deficiency is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

intravascular [acute] and extravascular, intrinsic, and inherited [XR]

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32
Q

clonal disorder with mutation in phosphatidylinositol glycan complementation group A (PIGA) gene

A

paroxysmal nocturnal hemoglobinuria

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33
Q

PNH causes decreased expression of GPI linked membrane glycoproteins

A

CD55 and CD59

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34
Q

PNH causes increased ____________________ lysis

A

complement mediated

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35
Q

PNH is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

intravascular, intrinsic, acquired

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36
Q

PNH demonstrates acquired mutations. This puts the patient at risk for

A

transformation to hematopoietic neoplasm

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37
Q

IgG antibodies recognized by splenic macrophages –> membrane loss

A

Warm autoimmune hemolytic anemia

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38
Q

warm hemolytic anemia occurs around what temperature

A

37 degrees C

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39
Q

what shape are the RBCs in warm hemolytic anemia?

A

spherocytes

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40
Q

warm autoimmune hemolytic anemia can be secondary to

A

infection
drugs
lymphoproliferative disorders

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41
Q

warm autoimmune hemolytic anemia is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

extravascular
extrinsic
acquired

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42
Q

IgM antibodies causing RBC agglutination and complement mediated lysis

A

cold autoimmune hemolytic anemia

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43
Q

cold autoimmune hemolytic anemia can be secondary to

A

lymphoproliferative disorders

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44
Q

cold autoimmune hemolytic anemia is [intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

intravascular and extravascular
extrinsic
acquired

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45
Q

antibody coating RBCs can be detected by

A

antiglobulin (Coombs) test

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46
Q

traumatic hemolytic anemia is
[intravascular/extravascular] [intrinsic/extrinsic] [inherited/acquired]

A

intravascular
extrinsic
acquired

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47
Q

prosthetic heart valves can cause

A

traumatic hemolytic anemia

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48
Q

trauma due to widespread clots in microcirculation

A

microangiopathic hemolytic anemia

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49
Q

pathology of traumatic hemolytic anemia

A

shistocytes
thrombocytopenia
variable abnormal coagulation tests

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50
Q

direct Coombs test

A

detects antibody coating in RBCs

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51
Q

indirect Coombs test

A

detects antibody in plasma that can react with RBCs

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52
Q

identifies IgM or IgG antibodies coating RBCs

A

direct antiglobulin test (DAT)

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53
Q

can differentiate etiology of spherocytes

A

direct antiglobulin test (DAT)

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54
Q

reaction to preformed ABO antibodies is mediated by

A

IgM

**intravascular hemolysis if ABO incompatibility

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55
Q

reaction of alloantibodies to other RBC antigens is mediated by

A

IgG

**extravascular hemolysis over weeks

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56
Q

2 steps of serological tests

A
  1. sensitization: abs binding to RBCs surface Ag
  2. agglutination: sensitized RBCs are bridged together to form the lattice
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57
Q

antiglobulin test (AGT)

A

bridging between sensitized RBCs is not strong enough to cause agglutination

AHG then used to enhance agglutination

**required for clinically significant Ab detection

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58
Q

fixes complement and causes intravascular hemolysis

A

IgM

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59
Q

large, with 10 potential antigen binding sites

A

IgM

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60
Q

binds to Ags on adjacent RBCs, forming lattice clump

A

IgM

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61
Q

“cold antibody”, reacts best at 22 degrees C in the IS

A

IgM

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62
Q

smaller, 2 antigen binding sites

A

IgG

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63
Q

binds to antibodies on single RBC, so unable to bridge

A

IgG

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64
Q

“warm antibodies” react at 37 degrees C in AHG

A

IgG

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65
Q

needs AHG reagent to see agglutination

A

IgG

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66
Q

Phase 1: Immediate Spin Phase (IS)

A

patients serum is mixed with suspension of commercial reagent RBCs

centrifuged and examined for agglutination

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67
Q

if agglutination is present in the IS phase

A

cold antibodies, IgM

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68
Q

Phase 2: in test tube

A

37 degree incubation to detect warm Abs

incubate and centrifuge

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69
Q

Phase 3: tube, solid phase, gel

A

Add AHG, incubate, centrifuge, examine for agglutination

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70
Q

each negative AHG tube must be followed by

A

a control of IgG coated red cells

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71
Q

most important single test in RBC ab detection

A

AGT, Coombs

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72
Q

detects patient’s RBC that have already been sensitized with IgG or complement in vivo

A

direct antiblobulin test (DAT)

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73
Q

detects anti-RBCs abs in patients serum
in-vitro coating of RBCs with antibody or complement

A

indirect antiglobulin test (IAT)

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74
Q

in gel testing, a positive reading is

A

agglutinated cells remain at top of column

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75
Q

in gel testing, a negative reading is

A

unagglutinated cells pellet at bottom of microtube

76
Q

in solid phase testing, a postive reading is

A

RBCs diffusely adhere over the well

77
Q

in solid phase testing, a negative reading is

A

indicated RBCs pellet to the bottom

78
Q

oligosaccarides of RBC Ag

A

ABO, I, P, MNS

79
Q

protein based RBC Ag

A

Rh, Kell, Kidd, Duffy, Diego

80
Q

a clinically significant all-Ab causes

A

hemolytic transfusion reaction (HTR)
hemolytic disease of fetus and enonate (HDFN)

81
Q

[warm/cold] reaction generally requires exposure

A

warm

82
Q

[warm/cold] reaction does not require exposure, is naturally occuring

A

cold

83
Q

terminal sugar of type A

A

galnac

84
Q

terminal sugar of type B

A

gal

85
Q

2 genes that encode Rh

A

RhCE and RhD

86
Q

P: IgG (auto Ab) is clinically significant for

A

paroxysmal cold hemoglobinuria

87
Q

Kidd is clinically significant for

A

intravascular hemolysis

88
Q

Forward Type:
Anti-A: +
Anti-B: 0

A

type A

89
Q

Forward Type:
Anti-A: 0
Anti-B: +

A

type B

90
Q

Forward Type:
Anti-A:0
Anti-B: 0

A

type O

91
Q

Forward Type:
Anti-A: +
Anti-B: +

A

type AB

92
Q

Ags on RBC detected by monoclonal antibody reagents at room temp

A

forward type

93
Q

testing patient’s serum with commercial RBC of known antigen types

A

reverse type

94
Q

Reverse Type:
A cells: 0
B cells: +

A

type A

95
Q

Reverse Type:
A cells: +
B cells: 0

A

type B

96
Q

Reverse Type:
A cells: +
B cells: +

A

type O

97
Q

Reverse Type
A cells: 0
B cells: 0

A

type AB

98
Q

checks for presence of unexpected non-ABO antibody in the recipients plasma

A

ab screen

**presence of alloantibodies from prior exposure

99
Q

cold antibodies are recognized in the ______phase

A

RT IS

100
Q

warm antibodies are recognized in the _______ or ______ phases

A

37 degrees
AHG

101
Q

an identification panel uses ___________________

A

group O reagent RBCs

102
Q

crossmatch is _____________ when transfusion is ______________

A

mandatory
not urgent

103
Q

if the ab screen is negative and there is no historical antibody the purpose of the XM is to

A

confirm ABO compatibility

104
Q

if the ab screen is negative and there is no historical antibody the method of XM is

A

electronic

105
Q

if the ab screen is negative and there is no historical antibody the XM takes how long?

A

5 min

106
Q

if there is history of Ab present, the purpose of the XM is to

A

confirm any incompatibility

107
Q

if there is history of Ab present, the method of XM is

A

complete XM

108
Q

if there is history of Ab present, the XM takes

A

45 min

109
Q

time required for ABO/Rh testing

A

10-15 min

110
Q

time required for type and screen

A

30-45 min

111
Q

time required for type, screen, and XM

A

45-60 min

112
Q

time required for antibody panel

A

100 minutes to days

113
Q

time required searching for untis

A

minutes to days

114
Q

the ultimate responsibility of following up on laboratory results is with

A

the ordering provider

115
Q

who is responsible for reporting critical results?

A

the lab performing the test

116
Q

Hb alpha is encoded for by

A

two identical a-globin genes on chr. 16

117
Q

how many copies of Hb alpha are in humans?

A

4

118
Q

Hb beta is encoded for by

A

a single B-globin gene n chr.11

119
Q

how many copies of Hb-beta are in humans?

A

two

120
Q

left shift on the oxygen-hemoglobin dissociation curve indicates

A

increased affinity for O2

121
Q

right shift on the oxygen-hemoglobin dissociation curve indicates

A

decreased affinity for O2

122
Q

O2 binding to Hb is ________

A

cooperative

123
Q

alpha and beta globin mutation causing reduction in amount of protein

A

thalassemai

124
Q

point mutation in B globin gene causing toxic gain of function by protein

A

sickle cell anemia

125
Q

deficiency from reduction in amount and/or activity of enzyme

A

G6PD deficiency

126
Q

severity of thalassemia depends on

A

severity of reduction in synthesis

127
Q

__________ of a-globin genes accounts for 80-85% of a-thalassemia

A

deletion

128
Q

____________ account for 90% of B-thalassemia

A

15 mutations

129
Q

B-thalassemia mutations can be caused by

A

splicing, gene promoter, non-sense/missense mutations

130
Q

severe disease, transfusion dependent, complete loss of at least one allele

A

B-thalassemia major

131
Q

severe disease but not transfusion dependet, intermediate reduction in amount of protein

A

b thalassemia intermedia

132
Q

limited or no disease, modest reduction in protein level

A

B thalassemia minor

133
Q

asymptomatic; 1 gene deleted

A

a-thalassemia silent

134
Q

asymptomatic or mild; 2 genes deleted

A

a-thalassemia trait

135
Q

severe disease; 3 genes deleted

A

a-thalassemia HbH disease

136
Q

lethal in utero; 4 genes deleted

A

a-thalassemia hydrops fetalis

137
Q

inheritance pattern of sickle cell anemia

A

autosomal recessive

138
Q

sickle cell: substitution of glutamate for val allows binding of a “pocket”. this causes

A

polymerization of tetramers and formation of long fibrils

139
Q

sickle cell anemia is caused by a misense mutation in

A

Hb beta gene

140
Q

sickle cell anemia is caused by a __________ mutation in the Hb beta globin gene

A

missense

141
Q

HbA

A

tetramer with wildtype alpha and Beta chains (a2b2)

142
Q

HbS

A

tetramer with mutated beta chains in sickle cell anemia

143
Q

sickle cell train

A

heterozygous

asymptomatic or very mild clinical phenotype

144
Q

inheritance pattern: G6PD deficiency

A

x linked recessive

145
Q

G6PD appears under _________

A

oxidative stress

(drugs or food)

146
Q

G6PD deficiency is caused by mutations that ___________ the protein

A

destabilize

**reduced activity

147
Q

usually asymptomatic until provoked

A

G6PD deficiency

148
Q

G6PD deficiency causes a reduction in __________, an ROS scavenger in RBCs

A

glutathione

**RBCs lack mitochondria to produce other reducing equivalents

149
Q

Primary way RBCs generate NADPH is via

A

pentose-phosphate pathway

150
Q

heterozygotes have increased fitness over homozygotes for the mutant or normal allele

A

balanced polymorphism

151
Q

increased fitness of heterozygotes causes

A

persistance of mutant allele in population

152
Q

mutations that allow balanced polymorphism against P. falciparum

A

alpha/beta mutations causing thalassemia
beta globin mutations causing sickle cell
G6PD deficiency

153
Q

mutations that allow balanced polymorphism against T. brucei

A

APOL1 allele associated with focal segmented glomerulosclerosis

154
Q

mutations that allow for balanced polymorphisms against diarrheal illnesses

A

CTFR allele causing cystic fibrosis

155
Q

racism and institutional barriers can make the management of _______________ complicated

A

sickle cell

156
Q

challenges to obtaining effective care for SCA

A

need for knowledgeable practitioner
racism
lack of efficient and evidence based management strategies

157
Q

prevention of sickling crisis

A

hydration
good nutrition
moderated exercise
minimize risks of low oxygen

158
Q

mainstay therapy of sickle cell anemia

A

hydroxyurea

159
Q

mechanism of hydroxyurea

A

ribonucleotide reductase inhibitor

*increases fetal Hb expression
*increased NO2
*decreases WBC/adhesion

160
Q

precursor for NAD+
likely reduces oxidative stress in sickling RBCs

A

L-glutamine

161
Q

allosteric inhibitor of HbS polymerization
binds alpha globin in HbS
increases O2 affinity

A

voxelotor

162
Q

pharmacotherapy for people >16 y.o. with crises unrelieved by hydroxyurea or L-glutamine

Monoclonal Ab against P-selectin

A

crizanlizumab

163
Q

gene therapy options for SCA

A

fix mutation with CRISPR-Cas9
express wildtype beta globin with gene transfer
reactivate fetal Hgb with CRISPR-Cas9

164
Q

treatment options for underlying cause of SCA

A

bone marrow transplant
gene therapy

165
Q

distinction between IDA and ACD is done through

A

iron studies

166
Q

a DAT test would identify which type of anemia

A

immune mediated

167
Q

hemoglobin electrophoresis is used to diagnose

A

hemoglobinopathy
thalassemias

168
Q

serum protein electrophoresis is used to evaluate

A

plasma cell neoplasms

169
Q

initial evaluation of thalassemia should include

A

HPLC of hemoglobin

looks for HbF or AbA2

170
Q

target cells are associated with

A

iron deficiency
thalassemia

171
Q

spherocytes are associated with

A

hereditary spherocytosis
autoimmune hemolytic anemia

172
Q

teardrop cells are associated with

A

bone marrow fibrosis

173
Q

hexagonal crystals

A

Hgb C

174
Q

elevation of HbA2 and HbF is consistent with

A

beta thalassemia

175
Q

elevated LDH
elevated serum bilirubin
decreased serum haptoglobin

A

think hemolytic anemia

176
Q

who establishes the threshold for critical values

A

healthcare organizations themselves

177
Q

bite cells

A

G6PD

178
Q

acquired PIGA mutations are associated with

A

PNH

179
Q

what can be done to decreases the severity of a sickling crisis?

A

decrease the proportion of HbS
-transfuse normal RBCs
-give drugs to enhance HbF production

180
Q

tetramers of beta globin chains

A

HbH

a thalassemia

181
Q

if a child inherits a bS gene from one parent and a b0 gene from another they will have

A

sickle cell

the b0 is nonfunctional, so the only beta globin produced will come from the bS gene

182
Q

if the patients screen is positive at AHG

A

probably have warm AIHA
proceed to 10 or 11 panel to identify

183
Q

forward type reaction with anti B indicates

A

Group B

184
Q

reverse type reaction with A1 indicates

A

anti A in plasma

sample is not group A blood

185
Q

reaction with anti D indicates

A

Rh positive

186
Q

among patients with sickle cell, which blood group system shows the greatest degree of genetic variation?

A

Rh

187
Q

allosteric inhibitor of HbS polymerization

A

voxelotor

IBI Test 3 (20 decks)

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