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B&L 1 > Anemia > Flashcards

Anemia Flashcards

1
Q

Anemia

A

insufficient red cell mass to adequately deliver oxygen to peripheral tissues

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2
Q

measurements to define anemia (8)

A 
hemoglobin concentration 
hematocrit
red blood cell count
MCV
MCHC
RDW
WBC count and differential
platelet count
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3
Q

percent volume of red cells in blood

A

hematocrit

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4
Q

variation in Hgb and Hct based on gender

A

higher than ever at birth
decrease to lower than adults in childhood
puberty go back to adult levels

menstruating women have lower values

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5
Q

How can we ID retics?

A

presence of mRNA

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6
Q

How are retics counted?

A

—as the percent of 1000 red cells counted (normal 0.4-1.7)

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7
Q

absolute retic count?

A

%retics x RBC count

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8
Q

reticulocyte index

A

ratio of how many fold beyond baseline the production of red cells is:

RI = Retic Count x (PatientHgb /Normal Hgb) x 1/stress factor

where 1.5 = mild
2 = moderate
2.5 = severe anemia

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9
Q

What should the RI be for a health individual?

A

1-2

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10
Q

2,3-DPG and anemia?

A

If develops over weeks, 2-3DPG compensatory mechanism will help O2 dissociate in tissue
If develops acutely, 2-3DPG does not have enough time to esatblish compensatory mechanism

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11
Q

Symptoms of anemia

A 
shortness of breath 
fatigue
rapid heart rate
dizzy
pain with exercise
pallor
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12
Q

signs of anemia

A

tachycardia
tachpnea
dyspnea
pallor

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13
Q

Question one in the classification of anemias

A

Are there any additional hematologic abnormalities

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14
Q

If anemia is associated with additional hemotologic abnormalities (e.g., thrombocytopenia, leukopenia, neutropenia) what should you consider?

A

look for infiltrative and proliferative processes (e.g., leukemia, lymphoma, aplastic anemia)

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15
Q

If the only manifestation is anemia, what should be your next question?

A

Is there an appropriate reticulocyte response to anemia?

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16
Q

If the only manifestation is anemia and there is an increase in reticulocytes, what should you consider?

A

increased red cell destruction (hemolysis) or hemorrhage

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17
Q

If the only manifestation is anemia and the retic count is not increased and there is no other evidence for hemolysis, what should you consider?

A

the type of anemia based on the MCV and size…i.e., normocytic, macrocytic, or microcytic

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18
Q

in what form does iron exist?

A

Iron exists in two valence states, ferric and ferrous - activity may depend on specific state

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19
Q

iron in aqueous solution

A

in aqueous solutions, iron forms insoluble hydroxides unless bound to a specific protein or other compound

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20
Q

at what pH is iron more soluble?

A

low

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21
Q

how does the body control iron balance?

A

controlled by absorption

there is no active excretion mechanism

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22
Q

iron losses?

A

losses each day are small

  • exfoliation of skin and mucosal surfaces (GI/skin)
  • in urine or with menstruation
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23
Q

is iron ever free in the body?

A

no

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24
Q

majority of iron is contained?

A

hemoglobin (65%)

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25
Q

primary storage form of iron?

A

ferritin and hemosiderin are the primary storage forms of iron (25% of the total body iron, mostly intracellular)

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26
Q

in addition to hemoglobin, what other oxygen binding protein binds iron and how much?

A

myoglobin (6%)

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27
Q

In addition to ferritin and hemosiderin, plus the oxygen binding proteins, what transport protein binds iron?

A

transferrin
a very small amount of iron is bound to transferrin, the transport protein which moves the iron to the tissues requiring iron - particularly the developing erythroid precursors.

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28
Q

Aside from the major iron binding proteins, what is the remaining

A

a whole variety of enzymes, including catalases, peroxidases, cytochromes, and other proteins which a critical to basic metabolic processes of the cell

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29
Q

Where does the absorption of iron take place?

A

mucosal surface of the duodenum

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30
Q

Hemoglobin iron form

A

Fe2+ (ferrous)

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31
Q

myoglobin iron form

A

Fe2+ (ferrous)

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32
Q

transferrin iron form

A

Fe3+ (ferric)

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33
Q

transferrin iron form

A

Fe3+ (ferric)

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34
Q

Plasma Fe transporter?

A

transferrin

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35
Q

Intracellular Iron sotarage?

A

Ferritin and hemosiderin

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36
Q

How do we get iron into mucosal cell?

A

DCYTB converts Fe3+ to Fe2+ then comes into cell through DMT1

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37
Q

Once iron is inside mucosal cell what happens?

A

Can be stored as ferritin
Or
Can cross basolateral membrane through ferroportin (Haphaestin coverts back to Fe3+)

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38
Q

what compound forms pore in basolateral membrane of mucous cell to allow mobilization of iron from cells into blood stream?

A

ferroportin

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39
Q

What compound produced by the liver decreases ferroportin?

A

hepcidin

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40
Q

types of iron absorbed?

A

elemental adn heme bound

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41
Q

Intraluminal factors influencing iron absorption (5)

A 
gastric (low pH / gastroferrin)
presence of proteins / amino acids (more)
vitamin c (more)
phytates / oxalates (less)
amount of iron ingested
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42
Q

Hephaestin

A

oxidizes Fe2+ to Fe3+ as it moves out of basolateral ferroportin to bind plasma transferrin

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43
Q

Extraluminal factors influencing iron absorption?

A

Erythropoietic activity (up)

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44
Q

Transferrin

A

main transport protein for iron (Fe3+)

binds 2 moles / mole

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45
Q

Where does transferrin go?

A

finds its way to the bone marrow and the maturing normoblasts where it binds transferrin receptors on the surface of cells where it is directed into cell and incorporated into hemoglobin

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46
Q

Who turns over erythrocytes?

A

macrophages in the spleen

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47
Q

what do macrophages do with the iron?

A

macrophages sequester iron in ferritin stores

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48
Q

is there extracellular ferritin?

A

only small amount

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49
Q

what happens to the ferritin bound iron in macrophages

A

evenutally iron from the storage pool may be released from teh cell and bound by transferrin again

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50
Q

what is the difference between ferritin and hemosiderin

A

in hemosiderin the iron is not completely soluble and bioavailble

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51
Q

what is hepcidin

A

25 aa antibacterial peptide produced by hepatocyte

negative regulator of iron absorption / transport / release

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52
Q

When do we make hepcidin

A

inflammation / infection / iron overload

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53
Q

hepcidin implication for hemochromatosis

A

deficiency of hepcidin

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54
Q

how does hepcidin increase iron retention in macrophage?

A

inhibits ferroportin - causes increase in macrophage retention - contributing to anemia

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55
Q

how does hepcidin influence iron resistant iron deficiency anemia?

A

implicated because iron won’t help if iron won’t be released

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56
Q

what is a negative regulator of iron absorption / transport / and release

A

hepcidin

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57
Q

Hepcidin release from hepatic cells during infection/inflammation –>

A

increased accumulation in macrophagic ferritin

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58
Q

splenic macrophage and iron?

A

stores Fe3+ in ferritin

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59
Q

Development of iron deficiency… first thing we see?

A

depletion of iron stores –> increase in iron absorption

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60
Q

Initial stage of iron deficiency, what is going on with transferrin?

A

should be the same saturation initially because we have only depleted stores - we are still going to be able to make normal amount of hemoglobin

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61
Q

Moderate stage of iron deficiency

A

We start to see a decrease in the serum as well as stores - then we see increase in iron binding capacity of transferrin and an incrase in iron absorption - start to see porphyrin rings that don’t have any iron in them (Protoporphyrin)

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62
Q

State of things when anemia sets in

A 
our iron binding capacity will be elevated
our ferritin will be low saturation
we will have increased absorption 
decreased serum iron 
increased protoporphyrin
micro/hypo erythrocytes
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63
Q

Hepcidin and ferroportin?

A

Hepcidin causes inhibtioion of ferroportin - leading to increased retention in macrophages contributing to anemia

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64
Q

Hepcidin is associated with which biological process?

A

inflammation/infeciton or iron overload

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65
Q

iron deficiency anemia is which kind?

A

micro/hypo

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66
Q

In addition to hematopoietic anemia, which other systems are affected?

A 
neuromuscular 
epithelial 
upper GI
Lower GI
Immune
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67
Q

IMicrocytic Iron deficiency anemia is most commonly seeni in?

A

Infants / Teen grils

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68
Q

Etiology of iron deficiency anemia (microcytic?)

A

decreased intake
increased loss
increased need

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69
Q

Iron deficiency anemia

Oxygen carrying capacity?

A

Hemoglobin and Hematocrit are down

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70
Q

Iron deficiency anemia

reticulocytes?

A

Decreased production :(

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71
Q

Iron deficiency anemia

MCV?

A

Microcytosis

Decrased MCV

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72
Q

Iron deficiency anemia

MCHC

A

Decrased

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73
Q

Iron Defieicney anemia

RDW

A

Increased

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74
Q

Iron deficiency anemia

Serum Fe?

A

Down

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75
Q

Iron deficiency anemia

TIBC

A

Increased

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76
Q

Iron deficiency anemia

ferritin level?

A

decreased

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77
Q

iron deficiency anemia

free erythrocyte proprophyrin (FEP)

A

Increased

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78
Q

Treatment of iron deficiency anemia

A

Oral iron

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79
Q

Normalization cascade iron deficiency anemia?

A

Serum iron –> Hbg/rectic –> ferritin –> MCV/FEP/RDW

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80
Q

Too much iron =

A

hemachromatosis

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81
Q

Etiology of hemachromatosis

A

too much in diet
too much absorption (HLA-H)
Repeat transfusions

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82
Q

Dangers of hemachromatosis

A

cardiac
liver
pancreas

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83
Q

serum iron
ferritin
liver iron
in hemachromatosis

A

all will be increased

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84
Q

treatment

hemochromatosis

A

therapeutic phleb

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85
Q

treatment

hemosiderosis

A

iron chelators

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86
Q

anemia of chronic disease (6) associated conditions

A 
chronic infection
chronic inflammation
malignant disease 
lead intoxication
renal insufficiency
endocrine issues
87
Q

pathophysiology of anemia in neoplasms and sepsis

A

increased TNF leads to decreased iron and EPO

increased INF-B

both lead to inhibition of erythroid proliferation and decreased RBC production

88
Q

pathophysiology of anemia in chronic infeciton and inflammation

A

increased IL1 release leads to decrease iron and decrease EPO

Increased INF-gamma

both lead to inhibition of erythroid proliferation and decreased RBC production

89
Q

pathophysiology of lead intoxication anemia

A

lead inhibits enzyme that puts iron on prophyrin ring and inhibits protoporphyrin synthesis

this lead to decreased heme + globin

90
Q

pathophysiology of renal insufficiency anemai

A

decreased EPO –> decreased erythroid proliferation

91
Q

We have a patient who comes in with fever, arthalgias, and fatigue…
Mild to moderate anemia (Hgb 8/12)
Normochromic, normocytic or microcytic with some hypochromia

They have decreased serum Fe
Decreased TIBC
normal to increased ferritin
decreased EPO for Hct
Decreased Rectic

What do you think they have?

A

Anemia due to chronic inflammation or infeciton

92
Q

What are distinguishing features of chronic inflammaiton or infeciton anemia?

A

Unlike iron deficiency, they will have decreased TIBC and normal to increased ferritin

93
Q

Chronic inflammation usually which type?

A

Normocytic or microcytic

94
Q

Chronic inflammation serum Fe?

A

down

95
Q

Chronic inflammation TIBC?

A

down

96
Q

Chronic inflammation ferritin

A

normal to increased

97
Q

chonic inflammation EPO?

A

down for Hct

98
Q

chronic inflammation retic count?

A

down

99
Q

Personality changes / irritaiblity / weight loss/ nausea etc are clinical features of which anemia?

A

lead toxicity

100
Q

lead toxicity anemia usually which type?

A

microcytosis and hypochromia

101
Q

what distinguishing features might we see with lead intoxciation anemia

A

increased zinc protoporphyrin

basophilic stippling

102
Q

what distinguishing features will we see in renal insufficiency anemia?
what type?

A

EPO deficiency

Normocytic

103
Q

Hypothyroidism, type?

A

most normochromic and normocytic, may be micro or macro

104
Q

Hyperthryoidism, type?

A

normo, may be micro

105
Q

When should we transfuse?

A

only transfuse red cells when the severity of anemia has potenital for cardiovascula decompensation

106
Q

when should we use EPO? (2)

A

an absolute deficiency
or
a decrease our of proportion to Hct, for which a response has been documented

107
Q

Sideroblastic anemia

A

underproduction

impaired protoporphyrin production or incorporation of iron

108
Q

accumulation of iron in mitochondria characteristic of

A

sideroblastic

109
Q

Folic acid and B12 are critical for synthesis of

A 
methionine from homocystiene 
which is needed for 
purine and pyrimidine biosynthesis
which are needed for
thymodylate for DNA synthesis
110
Q

what happens with Folate and B12 deficiencies (to cells)

A

Fucks with maturation

Cells increase in size and arrest in S phase –> destroyed –> ineffective erythropoiesis

111
Q

in addition to anemia, what else might we see with folate / B12 deficiency?

A

neutropenia

thrombocytopenia

112
Q

where is B12 absorbed?

A

terminal ileum

113
Q

what does B12 absorption require?

A

IF from gut

114
Q

What is the carrier of B12?

A

TC-II

115
Q

Where is folic acid absorbed?

A

jejunum

116
Q

What happens once folic acid is absorbed?

A

reduced/methylated

117
Q

Do B12 of folic acid stores last longer?

A

B12

118
Q

Causes of B12 deficiency? (5)

A 
Autoimmune
IF deficiency 
Malabsorption 
Defective transport / Storage
Metabolic defect
119
Q

Causes of folate deficiency? (6)

A 
dietary insufficiency
malabsorption 
drugs and toxins
inborn error of metabolism
increased demand (hemolysis/pregnancy/psoriasis)
increased loss or metabolism
120
Q

what type of anemia results from folate and vitamin b12 deficiencies

A

megaloblastic

121
Q

does folate or b12 deficiency develop more rapidly

A

folate

122
Q

is folate or b12 more likely to be associated with alcohol abuse / poor nutrition

A

folate

123
Q

is folate or b12 more likely to be associated with malabsorption

A

B12

124
Q

In folate and b12 deficiency what happens in bone marrow?

A

megaloblastic changes seen in both red cell and white cell precursors - at any stage, large, more immature nuclei

125
Q

In folate and b12 deficiency what happens with erythrocytes in bone marrow?

A

erythroid hyperplasia

126
Q

What happens with cytoplasmic maturation in folate and b12 deficiency?

A

normal

127
Q

in folate and b12 deficiency whats up with the peripheral blood?

A

MCV>97 - macrocytosis
ovalocytes
hypersegmented nuclei of neutrophils

128
Q

As anemia progresses in severity in folate and b12 deficient anemia what happens in peripheral blood?

A 
nuetropenia
thrombocytopenia 
increased bilirubin
LDH
RI
129
Q

Which anemia manifests neurologically

A

B12

130
Q

Good way to distinguish Folate vs B12?

A

A reaction involving vitamin
B12 but not folate is the synthesis of succinyl CoA from methylmalonyl CoA. Thus, in B12 but
not folate deficiency, methylmalonic acid levels are increased, making measurement of
methylmalonic acid a good way to distinguish the two.

131
Q

What fraction of B12 deficiency arise from GI?

A

95%

132
Q

Management of B12 deficiency?

A

1 mg injections weekly for first few weeks…then monthly

133
Q

How do we manage B12 if absorption not issue?

A

orally 2x/day

134
Q

How do we manage folate?

A

orally

135
Q

Folate / B12 anemia response to treatment?

A

reverses quickly

nuerological slower

136
Q

what is hemolysis?

A

decrease in red cell survival or increase in turnover beyond normal range

137
Q

how long do RBC take to develop in BM

A

10-14

138
Q

How long are reticulocytes in marrow?

A

3

139
Q

How long are retic in peripheral blood

A

1

140
Q

How long do RBC survive

A

120 +/- 20

141
Q

During stress what may happen to the time frame of reticulocyte release?

A

decrease BM maturation time to

5-7 days rather than 10-14

142
Q

Normal production of RBC (% red cell mass / day)

A

1

143
Q

How much may RBC production increase?

A

6-8 fold

144
Q

where does most RBC turnover take place?

A

spleen (extravascular 90%)

145
Q

where does minor RBC turnover take place?

A

intravascular 10%

146
Q

do we see changes in RBC enzyme activity with age?

A

yes this is normal

147
Q

do we see oxidative injury with RBC over time?

A

yes normal

148
Q

do we see changes in calcium balance?

A

yes normal

149
Q

do we see changes in carbohydrates and surface constituents?

A

yes, normal

150
Q

do we see antibodies to RBC surface constituents

A

apparently, but that’s weird

151
Q

extravascular RBC destruction is mediated by?

A

macrophages of the reticuloendothelial system

152
Q

in which type of hemolysis do red cells release hemoglobin into the circulation

A

intravascular

153
Q

what happens when hemoglobin is released into the circulation in intravascular hemolysis?

A

dissociates into dimer which may immediately bind to haptoglobin

haptoglobin is removed from circulation by liver

iron can be oxidized to form methemoglobin…dissociation of globin releases metheme which may bind to albumin or hemopexin - these can be taken up by parenchymal cells and converted to bilirubin

154
Q

if not dissociated to yield bilirubin, what alternative pathway may dimeric forms of methemoglobin / hemoglobin take in intravascular hemolysis?

A

filtered and not reabsorbed by kidney and appear in urine

155
Q

in which process are red cells ingestested by macrophages of the RE system

A

extravascular hemolysis

156
Q

what happens when RBC is ingested by macrophages in EVH?

A

the heme is separated from the globin, iron removed and stored in ferritin, and the porphyrin ring converted to bilirubin and released from the cell

157
Q

what happens to bilirubin that is released by macrophages?

A

taken up by transport system in the liver and converted to water soluble compound via conjugation of glucouronic acid

158
Q

what happens once glucouronic acid is conjugated to bilirubin in liver parenchymal cells?

A

secreted into the biliary tract and small bowel - the glucouronic acid is removed and bilirubin converted to urobilinogen and other water soluble pigments

159
Q

what happen with urobilinogen?

A

may cycle between teh gut and liver (entero-hepatic circulation) or excreted by the kidney into the urine…

160
Q

decrease in serum haptoglobin
hemoglobin in urin or plasma
increase in metheme / methemalubin
suugests?

A

intravascular hemolysis

161
Q

spectrin deficiency is most common abnormality seen in

A

hereditary spherocytosis

162
Q

hallmark of hereditary spherocytosis

A

loss of plasma membrane and formation of microspherocyte

163
Q

basic pathophysiology of hereditary spherocytosis

A

spectrin, ankyrin, or band 3 defects weaken the cytoskeleton and destabilize the lipid bilayer

164
Q

consequence of spherocyte formation?

A

decreased RBC deformability and entrapment in the spleen

165
Q

ultimate fate of RBC in hereditary spherocytosis

A

removal by macrophage

166
Q

clinical presentation of hereditary spherocytosis

A

variable degree of anemia as well as jaundice and splenomegaly -
1/3 has hyperbilirubinemia as neonates

167
Q

hereditary spherocytosis genetics

A

25% autosomal recessive

75% autosomal dominant

168
Q

splenectomy usually resolves clinical manifestations of which condition?

A

hereditary spherocytosis

169
Q

hereditary spherocytosis lab features

A 
variable Hct and Hgb
increased retic
decreased MCV
spherocytes on smear
unconjugated hyperhiliruinemia
170
Q

clinical complications of herediatry spherocytosis

A

aplastic crisis

bilirubin stones

171
Q

enzyme disorder anemias

A

G6PD

PK

172
Q

G6PD deficiency presents with

A

presents with hemolytic anemia

173
Q

G6PD genetics

A

X linked recessive

174
Q

G6PD enzyme

A

important enzyme in the pathway which provides protection again oxidant stress - loss of the enzyme actitvity in the red cells results in inability to restore reduced glutathione - with oxidant stress

175
Q

G6PD and oxidant stress –>

A

denatured hemoglobin attaches to the membrane and spectrin may be damaged - decreased deformability

176
Q

G6PD clinical presentation

A

intermittent episodes of acute hemolytic anemia and hyperbilirubinemia associated wtih oxidant stress

177
Q

G6PD characteristic cells

A

blister bite

178
Q

Pyruvate Kinase deficiency

A

decrease in converting phosphoenolpyruvate to pyruvate results in decreased ATP, increased 2,3-DPG, loss of membrane plasticity and increase in rigidity and destruction in the spleen

179
Q

PK deficiency clinical presentation

A

variable chronic anemia, hemolysis, increased reticulocytes, and no specific morphology

180
Q

Cold antibodies

A

activate complement

181
Q

Warm antibodies

A

incite splenic macrophage to antibody mediated phagocytosis through Fc receptor

182
Q

Warm or cold antibodies act via intravascular hemolysis?

A

cold

183
Q

warm or cold antibodies act via extravascular hemolysis?

A

warm

184
Q

direct antiglobulin test

A

evaluates for presence of IgG c3d or c4d on the surface of the patients red cells by addition of Coombs reagnet which has antibodies for IgG, C3d, and C4d causing agglutination

185
Q

indirect antiglobulin test

A

detect the ability of patient’s serum to bind IgG and or complement to test (normal) red blood cells - by definition, autoimmune hemolytic anemia should have a positive DAT

186
Q

Clinical characteristics of AIHA

A 
acute or chronic onset anemia
pallor
jaundice 
dark urine
splenomegaly may occur
187
Q

retic count in AIHA?

A

increased

188
Q

bilirubin in AIHA

A

increased

189
Q

is there hemoglobin in urine in AIHA?

A

Depends on the extent of intravascular hemolysis

190
Q

Which AIHA will exhibit psotiive DAT (strong IgG with weak complement)

A

warm

191
Q

which AIHA will exhibit positive DAT (complement only no IgG)

A

cold

192
Q

spleen is critical for

A

clearnace of intravascular microbes

193
Q

spleen important in children for development of

A

humoral response and is the origin of IgM agglutinins

194
Q

most significant complication of splenectomy?

A

bacterial sepsis associated with S pneumoniae

195
Q

risk of sepsis following splenectomy greatest in who

A

children under 5

196
Q

increased mortality from sepsis if splenectomy

A

200x

197
Q

pre-surgical splenectomy protocal

A

vaccination against
H influenza
S pneumoniae
meningococcus

198
Q

after splenectomy give?

A

penicllin

199
Q

most RBC turnover is extravascular occuring in the

A

spleen

200
Q

where does met-heme come from?

A

during intravascular hemolysis, iron is released from cell - converts to ferric form and yield met-heme which then binds albumin –> methemalbumin which is pulled out by the liver

201
Q

hemolytic anemia

RBC morphology

A

spherocytes / fragments

202
Q

hemolytic anemia

retic

A

increased

203
Q

bilirubin in hemolytic anemia

A

up because more Hgb is present to RE system - most unconjugated

204
Q

Hemolytic anemia

Hbg?

A

up

205
Q

hemolytic anemia

haptoglobin

A

low

206
Q

hemolytic anemia

methemalubin

A

up

207
Q

hemolytic anemia

housekeepign enzymes

A

up

208
Q

familial hereditary disorder characterized by anemia, intermittent jaundice, splenomegaly, and responsiveness to removal of spleen

A

herediatry spherocytosis

209
Q

most common molecular abnormality in Hereditary spherocytossi

A

spectrin

210
Q

we see abnormal response to hypotonic stress (osmotic fragility) in what disorder?

A

hereditary spherocytosis

211
Q

what is destablized in HS?

A

Lipid bilayer –> mkcrospherocytosis

212
Q

the loss of membrane surface area and subsequent microspherocytosis in HS leads to what consequence?

A

decreased RBC deformabiltiy and incrased entrapment in the splenic cords –> macrophage removal

213
Q 
lab features of HS
Hct?
Hgb?
Retic?
Mchc?
mcv?
A

vatiation in Hct and Hgb
retic up
mchc up
mcv down

also see spherocytes
unconjucated hyperbilirubinemia
increased osmotic fragility

B&L 1 (5 decks)

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