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PATHOMA > Ch. 5 - Microcytic anemia > Flashcards

Ch. 5 - Microcytic anemia Flashcards

1
Q

What is the MCV needed to have a microcytic anemia?

A
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2
Q

what actually causes the microcytosis?

A

an extra division of the erythroblast to maintain the proper Hgb concentration within the RBC

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3
Q

What are the four types of microcytic anemia?

A
  1. iron deficiency anemia2. anemia of chronic disease3. sideroblastic anemia4. thalessemia
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4
Q

Decreased levels of iron lead to decreased levels of (blank) which leads to decreased Hgb causing microcytic anemia

A

heme

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5
Q

What is the world’s most common nutritional deficiency?

A

iron def.

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6
Q

What form is the iron from vegetable derived forms

A

non-heme

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7
Q

Where in the GI tract does iron absorption ocurr?

A

duodenum

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8
Q

Is heme or non-heme iron more readily absorbed?

A

heme iron

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9
Q

(blank) transports iron from the gut lumen to the blood vessel

A

enterocytes

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10
Q

enterocytes use what protein to move iron into the blood?

A

ferroportin

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11
Q

What is iron bound to in the blood?

A

transferrin

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12
Q

what and where is iron bound in the storage form?

A

bound to ferritin in the liver and bone marrow macrophages

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13
Q

What does TIBC measure?

A

number of transferrin molecules in the blood?

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14
Q

what does % saturation measure? what is normal?

A

number of transferrin molecules bound to iron; 33% is normal

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15
Q

What does serum ferritin tell us?

A

reflects the levels of ferritin in the liver macrophages

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16
Q

What is the most common causes of iron def. anemia in infants?

A

breast feeding; low iron intake

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17
Q

What is the most common causes of iron def. anemia in children?

A

poor diet

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18
Q

What is the most common causes of iron def. anemia in adults 20-50?

A

peptic ulcer in males and menorrhagia in females (or pregnancy)

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19
Q

What is the most common causes of iron def. anemia in elderly?

A

Western world: colon polyps or carcinomaRest of world: hookworm

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20
Q

What two species of hookworm can cause iron def. anemia?

A

Ancylostoma duodonale and Necator americanus

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21
Q

How can malabsorption lead to iron def. anemia?

A

If you can’t take the iron in (lets say in Celiac), then you are def.

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22
Q

How can a gastrectomy cause iron def. anemia?

A

Gastric acid keeps iron reduced to the Fe2+ state; lower acid levels leads to the less available form of Fe3+

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23
Q

What are the clinical features of iron def?

A

Pica, anemia, and koilonychia

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24
Q

Describe the changes in RBC morphology in iron def. anemia?

A

increased RDW, hypochromic, microcytic

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25
Q

Describe the four steps of iron def. anemia progression

A
  1. Iron stores depleted2. Serum iron depleted3. Normocytic anemia4. Microcytic, hypochromic anemia
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26
Q

What two lab values identify that iron stores are depleted in the first stage of iron def. anemia?

A

Decreased ferritin and increased TIBC

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27
Q

What two lab values identify that serum iron is depleted in the second stage of iron def. anemia?

A

Decreased serum iron and decreased percent saturation

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28
Q

Why does iron anemia first present as normocytic before microcytic?

A

bone marrow is able to produce normal cells still, just an acceptably lower number of them

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29
Q

All together, what are the lab findings that support iron def. anemia?

A

decreased ferritinincreased TIBCdecreased serum irondecreased % saturation

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30
Q

How do you treat iron def. anemia?

A

ferrous sulfate

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31
Q

What is Plummer-Vinson Syndrome?

A

iron def. anemia with esophogeal webbing, atrophic glossitis, dysphagia, and BEEFY RED TONGUE

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32
Q

What type of anemia is associated with chronic inflammation?

A

Anemia of chronic disease (ACD)

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33
Q

What is the most common type of anemia in hospitalized pts?

A

ACD

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34
Q

Chronic disease leads to production of (blank) from the liver, including hepcidin

A

acute phase reactants

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35
Q

What are the two methods by which hepcidin sequesters iron?

A
  1. limit iron transfer from macrophages to erythroid precursors2. suppress EPO
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36
Q

What is the big picture reason why hepcidin hides iron in chronic inflammation?

A

body thinks its fighting bacteria, bacteria need Fe to grow

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37
Q

Why does ACD present as a microcytic anemia?

A

low iron means low heme means low Hgb means small cells

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38
Q

What are the following lab values in ACD?1. Ferritin2. TIBC3. Serum Iron4. % saturation5. FEP

A
  1. increased ferritin2. decreased TIBC3. decreased serum iron4. decreased % sat5. increased FEP
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39
Q

How do you treat ACD?

A

treat the underlying cause

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40
Q

Sideroblastic anemia is due to a defect in (Fe/protoporphyrin) useage

A

protoporphyrin synth

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41
Q

What is the rate limiting step of protoporphyrin synth?

A

Succ-CoA to ALA

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42
Q

What enzyme catalyzes the rate limiting step of protoporphyrin?

A

ALAS

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43
Q

What vitamin cofactor is necessary for the rate limiting step of protoporphyrin?

A

B6!!!

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44
Q

Where in the cell does Fe bind to protoporphyrin to make heme?

A

mitochrondria

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45
Q

in sideroblastic anemia, in what cellular structure is the iron sequestered?

A

mitochondria

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46
Q

t/F: sideroblastic anemia is only acquired

A

false; can be congenital from ALAS defect

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47
Q

What are the three major causes of acquired sideroblastic anemia?

A
  1. Alcoholism; mito poison2. Lead poisoning; inhibits ALAD and ferrochelatase3. Vit. B6 def.; seen as a SE from isoniazid Tx for TB
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48
Q

What are the following lab values in sideroblastic anemia?1. ferritin2. TIBC3. serum iron4. % saturation

A
  1. increased ferritin2. decreased TIBC3. increased serum iron4. increased % satruationIRON OVERLOADED STATE
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49
Q

What other liver disease has lab values similar to sideroblastic anemia?

A

hemachromotosis

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50
Q

in what cell type is the iron accumulating in sideroblastic anemia?

A

erythroblasts

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51
Q

How many alpha globulin chains are present on csome 16?

A

4

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52
Q

Is alpha-thal caused by gene deletion or mutation?

A

deletion

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53
Q

what happens when one alpha globulin gene is deleted?

A

pt. is asymptomatic

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54
Q

what happens when two alpha globulin genes are deleted?

A

mild anemia

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55
Q

What lab value is associated with a two alpha globulin gene deletion?

A

increased RBC count

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56
Q

Is the cis or trans deletion of two alpha globulin genes worse?

A

cis; offspring have high chance of severe anemia

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57
Q

In what racial pool do we see cis deletions in alpha-thal?

A

asians

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58
Q

in what racial pool do we see trans deletions in alpha-thal?

A

blacks

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59
Q

What form of Hgb is made when three alpha globulin genes are deleted?

60
Q

What is the globulin make up of HbH?

A

B4 tetramer

61
Q

What is the globulin make up of HbF?

62
Q

what is the globulin makeup of HbA?

63
Q

What is the globulin make up of HbA2?

64
Q

In three alpha globulin gene deletions, what shows up on electrophoresis?

65
Q

What is the effect of all four alpha globulin chains being deleted?

A

FATAL in utero aka hydrops fetalis; formation of g4 tetramers, damages RBCs

66
Q

Is b-thal due to gene mutations or deletions?

A

mutations; point in the promoter or splice sites

67
Q

in what racial pool do we see b-thal?

A

african and mediterranean descent

68
Q

How many b globulin genes are on c11?

69
Q

What is the name for a gene coding for no b globulin production and one coding for decreased globulin production?

A

none: B0low: B+

70
Q

What is the genotype of b-thal minor>?

A

B/B+ (one normal/one low)

71
Q

T/F: b-thal minor is usually asymptomatic

72
Q

what is the abnormal lab value associated with b-thal minor?

A

increased RBC count

73
Q

b-thal minor RBCs are microcytic, hypocrhomic, and (blank) in appearance

74
Q

WHat does electrophoresis of b-thal minor blood show?

A

decreased HbA with increased HbA2 (no beta chains) and HbF increased

75
Q

What is the genotype of b-thal major?

76
Q

when does the anemia present in b-thal major? why?

A

few months after birth; HbF is protective until the RBCs are degraded

77
Q

What damages the RBCs in b-thal major?

A

a4 tetramers that precipitate

78
Q

b-thal major causes (intra/extra)vascular hemolysis

A

extravascular hemolysis

79
Q

Massive erythroid hyperplasia in b-thal major can be seen by hematopoesis in what bones and (splemogealy/HSM)? What does this look like on XRAY?

A

skull and face bones; crewcut appearance and chipmunk facies, and HSM

80
Q

b-thal major pts are at risk of an aplastic crisis after infection with what virus?

A

parvovirus B19

81
Q

Chronic transfusions to treat b-thal major increases the risk for what disease?

A

secondary hemochromotosis

82
Q

b-thal major blood smears show microcytic, hypochromic RBCs with target cells and (blank other not normal cells)

A

NUCLEATED RBCs; holy fucking erythropoeisis, batman!

83
Q

Electrophoresis of b-thal major blood shows what?

A

HbA2 (no beta chains) and HbF with little to no HbA

84
Q

What is the normal reticulocyte count?

85
Q

How does retic count change in anemia?

A

falsely increased on the lab report

86
Q

How do you correct the retic count in anemic pts>?

A

(Actual Hct/45) x retic countExample: Hct 20%RETC: 15 %(20/45) x 15 = 6.6%

87
Q

(blank) requires an additional correction of (/2) to RETC

A

polychromasia

88
Q

What color do polychromatic cells appear on the smear?

A

light cyan blue

89
Q

Where is extramedullary hematopoiesis seen normally in babies and young children?

A

liver and spleen

90
Q

Generally speaking, where do newborn CBC values lie compared to infants and children?

A

higher than normal

91
Q

HbF shifts (blank) to the left

A

oxygen binding curve

92
Q

How long does it take for HbF to be replaced?

A

6-9 months

93
Q

What is the majority form of Hgb?

94
Q

In children, higher levels of phosphorus increase synth of 2,3 DPG leading to a (blank) shifted oxygen binding curve

A

right shifted

95
Q

What is the cutoff for anemia in adult males?

96
Q

what is the cutoff for anemia in adult females?

97
Q

Pregnancy leads to (lower/higher) normal ranges due to increased plasma volume

98
Q

What is the cutoff for anemia in pregnant women?

99
Q

How do you calculate MCV?

A

(Hct x 1000)/RBC count

100
Q

If your corrected retic count is greater than 3% in normocytic anemia, what does it mean?

A

means that the bone marrow response is EFFECTIVE in handling the anemia via erythroid hyperplasia

101
Q

If your corrected retic count is less than 3% in normocytic anemia, what does it mean?

A

means that the bone marrow response is INEFFECTIVE in hanlding the anemia via erythroid hyperplasia

102
Q

T/F: A corrected retic count greater than 3% means that the anemia is not caused by RBC defects

A

FALSE; caused by INTRINSIC or EXTRINSIC RBC defects

103
Q

How do you calculate MCHC?

104
Q

What does a low MCHC imply?

A

defect in Hgb synth and a microcytic anemia

105
Q

What does a high MCHC imply?

A

spherocytosis

106
Q

What is a variation in size called?

A

anisocytosis

107
Q

What does an increased RDW let you know?

A

can be seen in iron def. anemia; there are new cells being pumped out

108
Q

T/F: mature RBCs lack a mitochondria

109
Q

T/F: mature RBCs cannot perform the TCA cycle

110
Q

T/F: mature RBCs cannot perform B-ox of fats

111
Q

T/F; mature RBCs cannot perform the Pentose Phosphate pathway

A

FALSE; use it to reduce glutathione!

112
Q

T/F: mature RBCs cannot make ketone bodies

113
Q

T/F: mature RBCs cannot perform the Cori cycle

A

FALSE; that is anaerobic glycolysis and WHAT THEY RELY ON for energy

114
Q

What two cytokines drive ferritin synthesis?

A

IL1 and TNFa

115
Q

What does a decreased ferritin tell you?

A

DIAGNOSTIC OF IRON DEFICIENCY

116
Q

What does an icreased ferritin tell you?

A

ACD or iron overload, you know….since it is used for storage…got too much grain the silo, eh?

117
Q

Where is transferrin synthesized?

A

in the liver

118
Q

Where is ferritin synthesized?

A

macrophages of the bone marrow

119
Q

What is the normal serum iron level?

120
Q

TIBC correlates with what other lab value?

A

[Transferrin]

121
Q

What is the normal TIBC?

122
Q

What is the normal % saturation?

123
Q

How do you calculate percent saturation?

A

serum iron / TIBC x 100

124
Q

When ferritin goes down, TIBC (and transferrin therefore) goes (blank)

A

upTHEY HAVE AN INVERSE RELATIONSHIP

125
Q

Where is dietary iron resorbed? What percent is taken back up?

A

10% resorbed in duodenum

126
Q

What form of iron cannot be taken up in the gut>?

127
Q

What vitamin reduces Fe 3 + (ferric iron)?

128
Q

How much iron is stored in the bone marrow macs in men and women?

A

Men: 1 gwomen: 400mg

129
Q

Besides Hgb and in ferritin, what other molecules in the body use iron?

A

myoglobin and enzymes (used as cofactor)

130
Q

Achlorhydria (low levels of gastric acid) (inc/dec) availability of dietary iron

131
Q

What age groups and genders are at highest risk of iron def. anemia?

A

toddler’s 1-2females 12-49 from menstrual loss

132
Q

T/F: NSAIDS may cause iron def. anemia

A

true, via ulcers

133
Q

What named issue with the small bowel can lead to blood loss based iron def. anemia?

A

Meckel’s diverticulum

134
Q

What impact does prematurity have on Hgb levels?

A

inadequate and therefore iron def. anemia

135
Q

T/F: paroxysmal nocturnal hemoglobinuria is a type of iron def. anemia

136
Q

Iron def. anemia can change platelet counts how?

A

reactive thrombocytosis

137
Q

Do you treat B-thal?

138
Q

What is the common name for Cooley’s anemia?

A

b-thal major

139
Q

What chromosomal abnormality can lead to sideroblastic anemia?

A

X linked recessive (rare)

140
Q

What percent of hospitalized alcoholics have sideroblastic anemia?

141
Q

Whats another name for Vit. B6?`

A

pyridoxine

142
Q

What three enzymes does lead poisoning denature?

A
  1. ferrochelatase: binds Fe to protoporphyrin in the mito2. ALAD; increase in ALA3. ribonuclease: prevents breakdown of ribosomes
143
Q

Lead poisoning will denature ribonuclease leading to what finding on peripheral smear?

A

basophilic stippling

144
Q

What type of anemia is this?Abdominal colicEncephalopathy (due to increased δ–ALA)growth retardation (maybe not in Tommy boy’s case though)Peripheral neuropathyNephrotoxicityBurton’s line

A

sideroblastic

145
Q

(blank) is toxic to neurons, causes vascular permeability and edema and demyelination

146
Q

Do you see ringed sideroblasts in the peripheral smear or the bone marrow?

A

bone marrow; that’s where the iron is being made into Hgb and ultimately stored

PATHOMA (11 decks)

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