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Pathophys II - Exam 1 > Schoenwald > Flashcards

Schoenwald Flashcards

1
Q

symptoms of anemia

A

fatigue

tachycardia

HSM

dyspnea

pallor

bone pain

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2
Q

anemia is evaluated using what 2 values

A
  1. size → MCV
  2. hemoglobin concentration → MCHC
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3
Q

types of RBC morphology

A

spherocytes

schistocytes

target cells

tear drops

sickle cells

rouleaux

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4
Q

what are the 3 mechanisms by which anemia occurs

A
  1. decrease in RBC → acute or chronic
  2. decreased production of RBC
  3. increased destruction of RBC → hemolysis
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5
Q

what are the 4 mechanisms of anemia caused by increased RBC destruction

A
  1. external factors
  2. hereditary
  3. acquired defect
  4. paroxysmal nocturnal hemoglobinuria
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6
Q

anemia definition

A

decrease in RBC

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7
Q

what condition is caused by RBC becoming more prone to lysis dt a deficiency in CD55 and CD59

A

paroxysmal nocturnal hemoglobinuria

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8
Q

paroxysmal nocturnal hemoglobinuria is an __ disease

involving a defect in the __ gene

A

acquired

PIG-A

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9
Q

paroxysmal nocturnal hemoglobinuria results in __

A

venous thrombosis

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10
Q

paroxysmal nocturnal hemoglobinuria involves what 4 veins

A

hepatic

portal

mesenteric

cerebral

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11
Q

what is the cardinal symptom of paroxysmal nocturnal hemoglobinuria

A

episodic hemobloginuria of the 1st morning urine

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12
Q

increased destruction of RBC is broken into what 3 categories

A
  1. external factors
  2. hereditary internal factors
  3. acquired defects
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13
Q

name 3 microcytic anemias

A
  1. iron deficiency
  2. thalassemia
  3. anemia of chronic dz → late stage
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14
Q

in thalassemia, all iron labs are

A

normal

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15
Q

in IDA, __ and

__ are low,

and __ is elevated

A

low: ferritin, transferrin

high: TIBC

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16
Q

in the US, what are the 2 most common causes of IDA

A
  1. GI blood loss
  2. menstrual blood loss
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17
Q

outside the US, what is the most common cause of IDA

A

poor nutrition

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18
Q

IDA can also be caused by (2)

A
  1. malabsorption
  2. increased demand → pregnancy
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19
Q

thalassemia is caused by __

A

deficient production of Hgb

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20
Q

what are the 2 causes of decreased Hgb production in thalassemia

A
  1. mutation
  2. complete loss of one or more of the 4 copies of the alpha globulin chain
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21
Q

what are the 2 mechanisms by which RBC are removed from circulation

A
  1. extravascular
  2. intravascular
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22
Q

extravascular removal of RBC from circulation involves __

in the __

and __

A

phagocytosis

spleen and liver

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23
Q

what are 2 complications of extravascular RBC removal from circulation

A
  1. jaundice
  2. gallstones
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24
Q

intravascular removal of RBC from circulation involves

A

destruction of RBC in the vessel

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25
Q

what are 3 complications of intravascular removal of RBC from circulation

A
  1. tubular necrosis dt hemoglobinemia
  2. jaundice
  3. gallstones
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26
Q

what 3 lab values are increased in hemolysis

A

bilirubin

potassium

LDH

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27
Q

what 2 lab values are decreased in hemolysis

A

RBC

haptoglobin

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28
Q

in what 2 conditions will you see spherocytes

A
  1. hereditary spherocytosis
  2. immune hemolysis
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29
Q

in what 2 conditions will you see schistocytes

A
  1. TTP
  2. HUS
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30
Q

in what 2 conditions will you see target cells

A

hemoglobinopathies

  1. sickle cell anemia
  2. thalassemia
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31
Q

in what condition would you see teardrop cells

A

myelofibrosis

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32
Q

in what 2 conditions would you see sickled cells

A
  1. sickle cell anemia
  2. thalassemia minor w. sickle cell
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33
Q

in what condition would you see rouleaux anemia

A

multiple myeloma

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34
Q

what causes rouleaux

A

stacking of RBC due to increased globins or decreased albumin

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35
Q

what does increased reticulocyte indicate in terms of bone marrow

A

bone marrow is intact and able to produce RBC

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36
Q

in what 2 conditions would you see increased reticulocytes

A
  1. acute blood loss
  2. hemolysis
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37
Q

what does decreased reticulocyte count indicate in terms of bone marrow (2)

A
  1. primary bone marrow disorder

OR

  1. deficiency in building blocks
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38
Q

what are the 3 building blocks of RBC

A
  1. iron
  2. B12
  3. folate
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39
Q

what 3 labs are decreased in IDA

A
  1. ferritin
  2. serum iron (+/-)
  3. transferrin saturation
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40
Q

what 2 labs are increased in IDA

A
  1. TIBC
  2. RDW
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41
Q

what is transferrin saturation

A

ratio of serum iron to TIBC

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42
Q

what is nl for transferrin saturation

A

>20%

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43
Q

what is transferrin saturation in IDA

A

<10%

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44
Q

on a hgb molecule, there are __ copies of the alpha globulin chain

and __ copies of the beta globulin chain

A

4

2

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45
Q

in alpha thalassemia loss of one copy of the alpha globulin is called

A

silent (carrier)

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46
Q

in alpha thalassemia, loss of 2 copies of the globulin gene is called

A

trait → asymptomatic

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47
Q

alpha thalassemia trait is asymptomatic, but __ is low

A

MCV

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48
Q

in alpha thalassemia, loss of 3 copies of the alpha globulin chain gene is called

A

hemoglobin H disease (HbH) → marked anemia

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49
Q

in alpha thalassemia, loss of 4 copies of the alpha globulin chain gene is called

A

hydrops fetalis → stillborn

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50
Q

alpha thalassemia is prevalent in what 2 patient populations

A

Africans

Asians

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51
Q

does alpha thalassemia require a blood transfusion

A

no

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52
Q

beta thalassemia is characterized by a gene malfunction, resulting in

A

complete loss of the hemoglobin protein

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53
Q

beta thalassemia is prevalent in what patient population

A

mediterranean descent

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54
Q

what are 2 consequences of beta thalassemia when just alpha chains remain

A
  1. hemolysis
  2. ineffective erythropoiesis
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55
Q

beta thalassemia is categorized in to

A

major

minor

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56
Q

major thalassemia requires what type of tx

A

blood transfusion

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57
Q

usual onset of beta thalassemia is age __

and results in __

A

6 months

skeletal abnormalities

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58
Q

does beta thalassemia minor require a blood transfusion

A

no

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59
Q

what will you see in labs for beta thalassemia

A

disproportionate Hgb and MCV → 10/55

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60
Q

what 2 labs will be decreased in thalassemia

A

MCV

Hgb

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61
Q

what RBC morphology will you see in thalassemia

A

anisocytosis

poikilocytosis → target cells

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62
Q

what is anisocytosis

A

RBC of unequal size

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63
Q

what is poikilocytosis

A

abnormal shaped blood cells

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64
Q

name 4 conditions that can cause anemia of chronic dz

A
  1. lung carcinoma
  2. hodgkin lymphoma
  3. rheumatoid arthritis
  4. infxn → TB
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65
Q

anemia of chronic dz is initially __

but can become __ in later stages

A

normocytic

microcytic

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66
Q

what lab value is increased in anemia of chronic dz

A

ferritin

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67
Q

what 2 lab values are decreased in anemia of chronic dz

A

serum iron

TIBC

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68
Q

in anemia of chronic dz, __ and

__ cause erythropoietin to be decreased

A

TNF

interferons

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69
Q

in anemia of chronic dz, __ is stimulated,

which impairs release of iron from storage

A

hepcidin

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70
Q

what is hepcidin

A

a protein that regulates entry of storage iron into circulation

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71
Q

in IDA, __ (3) are decreased

and __ (1) is increased

A

decreased: ferritin, iron, transferrin saturation
increased: TIBC

72
Q

in anemia of chronic dz, __ (2) are decreased

and __ (2) are increased

A

decreased: iron, TIBC
increased: ferritin, transferrin saturation

73
Q

vitamin B12 deficiency causes __ DNA synthesis

involving __

A

impaired

all bone marrow precursors (not just RBC)

74
Q

impairment of DNA slows __ maturation

but not __ maturation

A

nuclear

cytoplasmic

75
Q

B12 is needed to regenerate what molecule

A

tetrahydrofolate

76
Q

what is tetrahydrofolate

A

the main active metabolite of dietary folate

77
Q

what is the function of tetrahydrofolate (folate)

A

DNA synthesis and repair

78
Q

why won’t folate supplementation effectively treat B12 deficiency

A

it will correct the anemia but not the neurologic symptoms bc B12 is needed to regenerate tetrahydrofolate

79
Q

aside from iron tests, what 2 elevations will you see in B12 deficiency

A
  1. homocysteine
  2. methyl-malonic acid (MMA)
80
Q

B12 deficiency causes anemia and what other two -penias

A

thrombocytopenia

leukopenia

81
Q

B12 deficiency causes ineffective __

and __ deficiencies

A

erythropoiesis

neurologic

82
Q

name 2 neurologic deficiencies that are seen in B12 deficiency

A
  1. peripheral neuropathy
  2. spinal cord pathology
83
Q

are neurologic deficiencies seen in folate deficiency

A

no!

84
Q

name 5 causes of B12 deficiency

A
  1. pernicious anemia
  2. nutrition
  3. gastrectomy
  4. ileal dz
  5. diphyllobathrium latum infxn (fish tapeworm)
85
Q

what are 2 causes of pernicious anemia

A
  1. abs against parietal cells or abs blocking B12 complex
  2. AI gastritis → loss of intrinsic factor
86
Q

list 4 clinical presentations of B12 deficiency

A
  1. pancytopenia
  2. loss of intestinal cells → diarrhea and malabsorption
  3. peripheral neuropathy
  4. hypersegmented neutrophils on CBC
87
Q

B12 deficiency causes __ neutrophils

A

hypersegmented

88
Q

folate deficiency is clinically indistinguishable from

A

B12 deficiency

89
Q

list 3 differences between folate deficiency and B12 deficiency

A
  1. no neurologic symptoms in folate
  2. folate is often dt dietary deficiency in US → etohism
  3. folate NOT associated w. pernicious anemia
90
Q

aplastic anemia is classified as

A

normocytic

91
Q

aplastic anemia is caused by absence of __

in the __

A

RBC

bone marrow

92
Q

what function is suppressed in aplastic anemia

A

stem cell fxn

93
Q

is there splenomegaly in aplastic anemia

A

no!

94
Q

what dz can aplastic anemia convert to

A

leukemia

95
Q

50% of anemia is

A

idiopathic

96
Q

aplastic anemia can be 2/2 to __ (5)

A

drug therapy

benzene

viral infxn

radiation

genetics

97
Q

all hemolytic anemias have increased (3)

A

RBC destruction

erythropoiesis

iron deposition in spleen and liver

98
Q

some hemolytic result in __ from increase in bilirubin

A

pigment gallstones

99
Q

what lab will be decreased in intravascular hemolytic anemia

A

haptoglobin

100
Q

what is haptoglobin and what is its fxn

A

protein produced by the liver

clears hgb

101
Q

why is haptoglobin decreased in hemolytic anemias

A

liver can not keep up w. hemolysis/production of new RBC → haptoglobin is cleared faster than we can make it

102
Q

what 2 lab values are elevated in intravascular hemolytic anemia

A

unconjugated bilirubin

LDH

103
Q

in intravascular hemolytic anemia urine will be positive for __

and __, which causes

__

A

hemosiderin

Hgb

→ causes brown urine

104
Q

extravascular hemolysis causes __ to be more increased than in intravascular,

but __

__ are less markedly affected

A

unconjugated bilirubin is more elevated

haptoglobin and LDH are less markedly affected

105
Q

extravascular hemolytic anemia is __ for hemosiderin and urine hgb

A

negative

106
Q

what are 3 congenital causes of hemolytic anemia

A
  1. G6PD deficiency
  2. cytoskeleton defects
  3. hemoglobinopathies
107
Q

what are 2 acquired causes of hemolytic anemia

A
  1. abs induced
  2. mechanical
108
Q

why does hemolytic anemia cause increased unconjugated bilirubin

A

unconjugated bilirubin is a breakdown product of hgb

hemolytic anemia causes destruction of hbg → increased unconjugated bilirubin

109
Q

what is meant by intravascular hemolysis

A

RBC destroyed within the blood vessel

110
Q

what is meant by extravascular hemolysis

A

RBC destroyed in hepatic and splenic macrophages

111
Q

direct and indirect coombs are __ tests

A

antiglobulin

112
Q

in the direct coombs test looks for abs located

A

on the surface of the RBC

113
Q

the indirect coombs test looks for abs located

A

in the bloodstream

114
Q

what type of coombs test is used to detect hemolytic anemia

A

direct

115
Q

which type of coombs test is used to indicated a bad rxn to a transfusion

A

indirect

116
Q

name 3 conditions in which coombs testing may be indicated

A
  1. autoimmune hemolytic anemias
  2. erythroblastosis fetalis
  3. lupus
117
Q

name 4 types of antibody mediated destruction of RBCs

A
  1. incompatible RBC transfusion rxns
  2. erythroblastosis fetalis
  3. warm and cold autoimmune anemias
118
Q

a positive result in coombs testing is indicated by

A

agglutination

119
Q

ABO transfusion rxns are __ mediated and involve

__ hemolysis

A

IgM

intravascular

120
Q

isohemaglutinins are produced in what type of rxn

A

incompatible RBC transfusion rxns

121
Q

erythroblastosis fetalis is also called

A

hemolytic dz of the newborn

122
Q

erythroblastosis fetalis results from __ incompatibility

A

Rh

123
Q

what causes the renal failure and high mortality rate in ABO transfusion reactions

A

hemoglobinemia

124
Q

anti a and anti b IgM’s are naturally occurring in what population

A

O blood type

125
Q

non ABO transfusion reactions involve __ antibody

and __ hemolysis

A

IgG

extravascular

126
Q

in non ABO transfusions, the antibody first binds to the __

and then this complex is removed by the __

A

RBC

spleen

127
Q

non ABO rxns involve previous exposure to

A

non ABO antigens (ex. Rh factor)

128
Q

what is a clinical manifestation of a non ABO transfusion rxn

A

jaundice

129
Q

which Rh antigen has the most potent rxn in erythroblastosis fetalis

A

RhD

130
Q

in erythroblastosis fetalis, if the mom is D (-) and the child is D (+), what is the outcome

A

the child is ok

131
Q

in the second child, if the mom is D (-) and the child is D (+), what happens

A

IgG is present in mom, causing fetal RBC hemolysis

132
Q

name 3 clinical presentations of erythroblastosis fetalis

A
  1. hydrops fetalis
  2. severe unconjugated hyperbilirubinemia
  3. kernicterus
133
Q

what is hydrops fetalis

A

severe fetal anemia

134
Q

what is kernicterus

A

mental retardation caused by high levels of bilirubin in a baby’s blood

135
Q

what is the preventive tx for erythroblastis fetalis

A

MOC is given Rhogam (anti D antibody) during pregnancy

136
Q

which type of coombs test is used in erythroblastosis fetalis

A

direct coombs test

137
Q

in Warm Autoimmune Anemia, there is an __

vs __ rxn

A

IgG vs self

138
Q

why is it called “warm” autoimmune anemia

A

the IgG vs self rxn occurs at body temp (37C)

139
Q

why is warm autoimmune anemia called an opsonin like rxn

A

opsonin tags cells for phagocytosis

in warm autoimmune anemia autoantibodies tag RBC for phagocytosis

140
Q

what type of hemolysis is warm autoimmune anemia

A

extravascular

141
Q

what are 3 clinical symptoms of warm autoimmune anemia

A
  1. anemia
  2. jaundice
  3. +/- splenomegaly
142
Q

in warm autoimmune anemia, __ coombs test will be positive

and retic count will be __

A

direct

elevated

143
Q

primary warm autoimmune is also called __

and accounts for __ of cases

A

idiopathic

60%

144
Q

name 3 causes of secondary warm autoimmune anemia

A
  1. drugs
  2. B cell neoplasm
  3. lupus
145
Q

in cold autoimmune hemolytic anemia IgM binds to RBC at __ degrees

and causes the cleavage of __ RBC

A

30C

C3b RBCs

146
Q

name 2 causes of acute autoimmune hemolytic anemia

A
  1. mycoplasma PNA
  2. EBV
147
Q

name 2 causes for chronic cold autoimmune hemolytic anemia

A
  1. idiopathic
  2. B cell neoplasms
148
Q

in cold autoimmune hemolytic anemia, __ coombs test

is __

A

direct

negative

149
Q

what type of RBC morphology is associated with cold autoimmune hemolytic anemia

A

spherocytes

150
Q

mechanical valves, ITP, TTP, DIC, malaria, and Babesia are all examples of what type of anemia

A

hemolytic

151
Q

name 3 categories of hereditary destruction of RBC

A
  1. defect in cytoskeleton
  2. structurally abnormal hemoglobins
  3. enzyme deficiency
152
Q

hereditary spherocytosis is caused by a defect in the __ of the RBC

A

cytoskeleton

153
Q

in hereditary spherocytosis, the RBC becomes less __

and is trapped in the __

A

flexible

spleen

154
Q

which type of coombs test is used in hereditary spherocytosis, and what is the result

A

direct coombs

negative

155
Q

name 2 other tests besides coombs that are associated with hereditary spherocytosis

A
  1. increased osmotic fragility test
  2. increased unconjugated bilirubin
156
Q

name 2 clinical signs of hereditary spherocytosis

A
  1. jaundice
  2. splenomegaly
157
Q

what is the tx for hereditary spherocytosis

A

splenectomy

158
Q

what is homozygous sickle cell anemia

A

all normal hemoglobin is replaced with Hbs (sickled Hgb) → more severe form of sickle cell anemia

159
Q

what is heterozygous sickle cell anemia

A

50% of normal hemoglobin is replaced with Hbs (sickled hgb)

160
Q

heterozygous sickle cell anemia is considered

A

asymptomatic

161
Q

sickle cell anemia disproportionately affect what patient population

A

African Americans

162
Q

heterozygous sickle cell anemia (trait) is protective against what other disease

A

malaria

163
Q

Hbs (sickled Hgb) is characterized as __

and is made worse by what 2 factors

A

sticky

altitude and dehydration

164
Q

name 6 complications of sickle cell anemia

A
  1. microvascular obstruction → ischemia and infarction
  2. emboli of necrotic bone marrow -→ acute chest syndrome
  3. autosplenectomy → increased risk of infxn
  4. priapism (persistent painful erection), cerebral infarcts
  5. increased erythropoiesis
  6. renal and pulmonary failure
165
Q

G6PD deficiency is an X linked __ disease

A

recessive

166
Q

what is the most common enzyme deficiency to cause hemolysis

A

G6PD deficiency

167
Q

what 2 pt populations does G6PD deficiency-enzyme favor

A

middle eastern

african

168
Q

in G6PD deficiency, the body is unable to regenerate __,

which helps reduce oxidative stress

A

glutathione

169
Q

name 6 oxidative factors that can exacerbate G6PD deficiency

A
  1. antimalarial (primiquine)
  2. sulfonamides
  3. ASA
  4. nitrofurantoin
  5. viral hepatitis
  6. fava beans
170
Q

what are 2 microscopic findings associated w. G6PD deficiency

A

heinz bodies

bite cells

171
Q

what do you think of when you see: red face, HA, hgb > 60%

A

polycythemia

172
Q

how does polycythemia affect RBC

A

increases number of RBC

173
Q

relative polycythemia is caused by

A

reduced plasma volume → hemoconcentration

174
Q

primary polycythemia is also called

A

polycythemia rubra vera

175
Q

polycythemia rubra vera is a type of __

that causes proliferation of __

A

blood cancer

RBC

176
Q

name 3 causes of secondary polycythemia

A
  1. lung dz
  2. cyanotic heart dz
  3. erythropoietin producing tumors

Pathophys II - Exam 1 (4 decks)

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