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LAST CLASS > Lec 7 & 8 > Flashcards

Lec 7 & 8 Flashcards

1
Q

problem with intrinsic hemolytic anemia

A

problem with RBC

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2
Q

problem with extrinsic hemolytic anemia

A

problem outside RBC

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3
Q

what % of RBC destruction is extravascular (normal conditions)

A

80%

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4
Q

what % of RBC destruction is intravascular (normal conditions)

A

10 to 20%

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5
Q

what will happen to unconjugated/indirect bilirubin during excessive extravascular hemolysis

A

increase

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6
Q

what will happen to urobilinogen during excessive extravascular hemolysis

A

increase

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7
Q

extravascular hemolysis: end product heme

A

bilirubin

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8
Q

where does extravascular hemolysis occur in the body

A

spleen or liver

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9
Q

what will be increased in urine during excessive extravascular hemolysis

A

urobilinogen

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10
Q

what is present in the blood vessels when RBCs are lysed intravascularly.

A

free Hb

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11
Q

what does free Hb cause in the urine

A

hemoglobinuria

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12
Q

T or F: free Hb is not toxic

A

F

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13
Q

what happens to the kidney when free Hb goes there

A

it loses iron

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14
Q

what blood protein binds to free Hb

A

haptoglobin

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15
Q

haptoglobin/hemopexin ____ when intravascular hemolysis increases

A

decreases

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16
Q

what pathological processes can cause an increase in intravascular hemolysis

A

sickle cell disease, hemolytic transfusion rxns, and sepsis

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17
Q

what causes gallstones in severe anemia

A

the super saturation of bile

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18
Q

what is a symptom of hereditary spherocytosis

A

gallstones

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19
Q

def? decrease urine production

A

oliguria

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20
Q

def? no urine production

A

anuria

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21
Q

what causes methemoglobinuria in severe anemia

A

increased amount of methemoglobin in the blood

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22
Q

describe the retic count, HCT, RBC count in hemolytic anemia

A

reticulocyteosis, decreased HCT, decreased RBC count

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23
Q

what happens to LDH and HbA1C values during accelerated RBC destruction

A

increased

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24
Q

what is another name for intravascular hemolysis

A

fragmentation

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25
Q

what is another name for extravascular hemolysis

A

macrophage mediated hemolysis

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26
Q

what test help differentiate between intra and extravascular hemolysis

A
  • haptoglobin
  • free hb
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27
Q

what is seen on the PBS of intravascular hemolysis

A

schistocytes

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28
Q

what is seen on the PBS of extravascular hemolysis

A

spherocytes

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29
Q

disease?
- polychromasia
- spherocytes
- nRBCs

A

hemolytic anemia

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30
Q

what will hemolytic anemia cause the BM to look like

A

erythroid hyperplasia

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31
Q

what are the RBC ezymopathies of hemolysic anemia

A

G6PD def and PK def

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32
Q

what type of hemolytic anemia is hereditary spherocytosis (HS)

A

chronic

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33
Q

how are spherocytes formed

A

vertical mmb protein def

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34
Q

how does hereditary spherocytosis get inherited

A

autosomal dominant

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35
Q

what RBC mmb protein are involved with vertical mmb interactions

A
  • spectrin
  • ankyrin
  • band 3
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36
Q

what are the clinical features of HS

A

anemia, jaundice, splenomegaly, and gallstones

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37
Q

HS: ____ MCHC, ____ retic count, direct antiglobin test _____, _____ haptoglobin, ____ LDH, and ____ unconjugated bilirubin test

A

increased, increased, neg, low, high, increased

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38
Q

immune hemolytic anemia will always have a ____ direct antiglobin test

A

pos

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39
Q

treatment for HS

A

splenectomy or blood transfusion

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40
Q

what happens to osmotic fragility in HS

A

increased

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41
Q

what happens to target cells osmotic fragility

A

decreased

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42
Q

when spherocytes are present EMA will be _____ because _______ mmb proteins are mutated

A

decreased, vertical

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43
Q

how is hereditary elliptocytosis (HE) inherited

A

autosomal dominant

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44
Q

what is the severe form of HE called

A

hereditary pyropoikilocytosis (HPP)

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45
Q

how are elliptocytes formed

A

horizontal mmb proteins are mutated

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46
Q

what are the horizontal RBC mmb proteins

A

cytoskeleton, alpha-spectrin, beta-spectrin, or protein 4.1

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47
Q

symptoms of HE

A

most people are asymptomatic

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48
Q

how can you tell if someone is autosomal homozygous for HE

A

100% elliptocytes in PBS

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49
Q

how is HPP inherited

A

autosomal recessive

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50
Q

what other disease has a similar PBS to HPP

A

thermal burns

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51
Q

why will MCV be low in HPP

A

RBC fragments present

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52
Q

RBC from HPP pt will be very sensitive to what

A

heat

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53
Q

how are stomatocytes formed

A

defect with pump on RBC mmb

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54
Q

how is hereditary stomatocytosis inherited

A

autosomal dominant

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55
Q

is paroxysmal nocturnal hemoglobinuria acquired or inherited

A

acquired

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56
Q

what gene is mutated with paroxysmal nocturnal hemoglobinuria

A

genes that code for CD55 and CD59

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57
Q

what is the normal function of CD55 and CD59

A

protect RBC against compliment system

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58
Q

what color urine of paroxysmal nocturnal hemoglobinuria

A

dark

59
Q

RBC phenotype of paroxysmal nocturnal hemoglobinuria: normal marker level, little or no complement-mediated hemolysis

A

RBC I

60
Q

RBC phenotype of paroxysmal nocturnal hemoglobinuria: partial def of CD55 and CD59, and these cells are relatively resistant to complement-mediated hemolysis

A

RBC II

61
Q

RBC phenotype of paroxysmal nocturnal hemoglobinuria: no CD55 and CD59 proteins, highly sensitive to spontaneous lysis by complement

A

RBC III

62
Q

how is G6PD def inherited

A

X linked

63
Q

what class of G6PD def is the most severe

A

I

64
Q

common PBS finding in G6PD def

A

heinz body

65
Q

what stain is used for Heinz bodies

A

supra vital stain

66
Q

what clinical syndromes cause G6PD def (3)

A

anti-malarial drugs, infection, fava bean

67
Q

hemolytic anemia will be ____cytic and ____chromic

A

normo; normo

68
Q

what RBC morphology will confirm G6PD def

A

Heinz bodies

69
Q

what will the retic count be in G6PD def

A

very high

70
Q

what is the function of PK

A

generates 1 mol of ATP in RBC glycolysis

71
Q

what happens when people are PK def

A

their RBCs can not produce enough ATP

72
Q

what will be seen on the PBS of a pt with PK def

A

burr cells

73
Q

what kind of anemia does a PK def cause

A

chronic hemolytic anemia

74
Q

how is PK def inherited

A

autosomal recessive

75
Q

what is the 2nd most common cause of enzyme-def hemolytic anemia

A

PK def

76
Q

what cell is seen in MAHA PBS

A

schistocytes

77
Q

what kind of hemolysis is involved in MAHA

A

mostly extravascular

78
Q

what are the associated disorders with MAHA

A
  • TTP
  • HUS
  • DIC
  • HELLP
79
Q

what is the problem with TTP

A

decrease in plt count

80
Q

what enzyme is mutated in TTP

A

ADAMTS13

81
Q

what is the normal funtion of ADAMTS13

A

Degrades von willebrand factor

82
Q

what is the best best treatment for TTP

A

plasma exchange

83
Q

what causes HUS

A

bacterial infection

84
Q

what is another name for HUS

A

Hamburger disease

85
Q

what type of people are most likely to get HUS

A

children

86
Q

what is produced in HUS that destroys cells

A

Shiga toxin

87
Q

what will be seen in the PBS of HUS

A
  • RBCs fragments
  • throbytopenia
88
Q

what organ is effected in HUS

A

kidney

89
Q

what kind of microorganisms is malaria

A

intracellular

90
Q

what is the vector of malaria

A

female anopheles mosquito

91
Q

what type of malaria lifecycle happens in humans

A

asexual

92
Q

(malaria) when the mosquito 1st bites you where does the parasite go

A

liver

93
Q

what stage of malaria is in the liver cells

A

schizont

94
Q

how do mosquitos get infected with malaria

A

eats blood with gametocytes

95
Q

how many blood smears are made for malaria testing

A

2 (thin and thick)

96
Q

what type of microorganism is babesia

A

intracellular

97
Q

what is the vector of babesia

A

ixoodes scapularies (tick)

98
Q

what lab test is used to diagnosis babesia

A

indirect immunofluorescent Ab assay

99
Q

what are the 3 types of autoimmune hemolytic anemia

A
  • warm- reactive ab
  • cold-reactive ab
  • PCH
100
Q

what is another name for DAT test

A

direct Coombs test

101
Q

t or f: warm autoimmune hemolytic anemia is non spherocytic hemolytic anemia

A

t

102
Q

what will be seen on the PBS of warm autoimmune hemolytic anemia

A
  • polychromasia
  • spherocytes
103
Q

what temp do warm reactive ab react at

A

body temp

104
Q

what type of ab is involved in warm autoimmune hemolytic anemia

A

IgG

105
Q

is autoimmune hemolytic anemia DAT pos or neg

A

pos

106
Q

what type of hemolysis occurs in autoimmune hemolytic anemia

A

extravascular

107
Q

cold autoimmune hemolytic anemia: MCV falsely ___, WBC falsely ___, and RBC falsely ____

A

high, high, low

108
Q

what type of cold reactive ab are present in cold autoimmune hemolytic anemia

A
  • monoclonal cold agglutinins
  • IgM
109
Q

autoimmune hemolytic anemia: when does IgM bind to RBCs

A

during cold temps

110
Q

what do IgM autoantibodies activate

A

complement

111
Q

autoimmune hemolytic anemia: when does IgM ab dissociate

A

when RBC return to central circulation

112
Q

autoimmune hemolytic anemia: what will still remain on RBC once IgM ab dissociates

A

C3b

113
Q

what does DAT detect

A

C3b

114
Q

what type of hemolysis happens in autoimmune hemolytic anemia

A

extravascular

115
Q

autoimmune hemolytic anemia: ___ titer at 40 C

A

high

116
Q

what is seen on the PBS of autoimmune hemolytic anemia

A

agglutination

117
Q

what must be done to a blood sample that is being tested for autoimmune hemolytic anemia

A

pre warmed

118
Q

def? acute form of cold reactive hemolytic anemia

A

paroxysmal cold hemoglobinuria

119
Q

paroxysmal cold hemoglobinuria is a secondary condition to what

A

viral infections in kids

120
Q

what ab is involved in paroxysmal cold hemoglobinuria

A

anti-P autoantibody

121
Q

what ab is biphasic

A

anti-P autoantibody.

122
Q

when does P ab bind to P ag

A

cold temps

123
Q

t or F: when P ab binds to P ag at cold temps it completely activates complement

A

F

124
Q

when is complement completely activated in paroxysmal cold hemoglobinuria

A

when anti- P autoantibody goes to body core

125
Q

what is another name for anti-p autoantibody

A

donath-landsteiner ab

126
Q

what is seen on the PBS of paroxysmal cold hemoglobinuria

A

erythrophagocytosis

127
Q

def? a small molecule which, when combined with a larger carrier such as a protein, can elicit the production of antibodies which bind specifically to it

A

hapten

128
Q

what kind of drug is penicillin

A

hapten

129
Q

what type of drug-induced hemolytic anemia: drug binds to RBC mmb –> ab binds to drug –> RBC is lysed

A

hapten mechanism

130
Q

what type of drug-induced hemolytic anemia: combo of drug and Ab causing RBC to lyse

A

autoimmune mechanism

131
Q

what is the common autoimmune mechanism drug

A

methyldopa (hypertension drug)

132
Q

what type of drug-induced hemolytic anemia: drug changes the structure of protein –> makes a new Ag –> that ag bind to RBC —> RBC lyses

A

immune complex mechanism

133
Q

what Is a the common immune complex mechanism drug

A

anti-malaria drugs

134
Q

if a hemolytic transfusion rxn is immediate what type of ab is present

A

IgM

135
Q

if a hemolytic transfusion rxn is delayed what type of ab is present

A

IgG

136
Q

what type of ab is Rh ab

A

IgG

137
Q

t or f: Hb doesn’t rise in delayed hemolytic transfusion rxn

A

T

138
Q

in HDN the mom will be Rh ___ and the baby will be Rh ____

A

neg , pos

139
Q

in HDN what kind of sample will be taken to test for Ab D

A

serum

140
Q

what drug is given to moms to prevent HDN

A

RhoGAM

141
Q

HDN: high titer signals

A

Ab D is present

142
Q

what test is used to test for HDN

A

kleihauer-betke acid elution

143
Q

kleihauer-betke acid elution: fetal cells will be ____ color because they contain Hb ___

A

pink, F

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