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Red Blood Cell Disorders Flashcards

1
Q

Define anemia

A

Reduction in circulating red blood cell mass

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2
Q

What symptom does anemia present like?

A

Hypoxia

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3
Q

Is anemia of pregnancy actually anemia?

A

No, just increased plasma volume

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4
Q

Symptoms of hypoxia/anemia include? (4)

A
  1. Weakness/fatigue/dyspnea
  2. Pale conjunctiva and skin
  3. Headache and lightheadedness
  4. Angina
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5
Q

What measurements are used to determine RBC mass?

A
  1. RBC count
  2. Hemoglobin
  3. Hematocrit
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6
Q

How is anemia defined in terms of Hb?

A

Males: Less than 13.5
Females: Less than 12.5

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7
Q

How is anemia defined in terms of MCV?

A

Microcytic: Less than 80
Normocytic: 80-100
Macrocytic >100

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8
Q

Anemias due to red cell production decrease are due to what? (2)

A
  1. Hematopoietic cell damage

2. Deficiency of factors for heme synthesis of DNA synthesis

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9
Q

Anemias due to increased red cell loss are due to what? (2)

A
  1. External blood loss

2. Red cell destruction

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10
Q

What is acute posthemorrhagic anemia?

What are clinical symptoms similar to

A

Following acute blood loss, there may be no decrease in any RBC mass markers but an increase in platelet count.

Hypovolemia

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11
Q

What are the four microcytic anemias?

A
  1. Iron deficiency
  2. Anemia of chronic disease
  3. Sideroblastic anemia
  4. Thalassemias
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12
Q

Microcytic anemia has what MCV?

A

Less than 80

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13
Q

Microcytic anemias are simply the result of what?

A

Decreased production of hemoglobin

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14
Q

Why does the microcytosis occur in microcytic anemias?

A

Extra cellular divisions to make smaller cells with same hemoglobin concentration as normal RBC’s.

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15
Q

Hemoglobin is made of what?

A

Heme and globin

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16
Q

Heme is made of what?

A

Iron and protoporphyrin

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17
Q

A decrease in what substances will result in a microcytic anemia? (3)

A
  1. Globin
  2. Iron
  3. Protoporphyrin
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18
Q

What is the most common type of anemia?

Why?

A

Iron-deficiency anemia

Most common nutritional deficiency in the world.

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19
Q

Absorption of iron occurs where?

A

Duodenum

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20
Q

What are the two forms iron is absorbed in?
Which is absorbed more readily?
What are the transporters for each

A
  1. Heme form (from meat): Absorbed quicker
  2. Non heme form (from vegetables)
  3. Heme: Heme transporter
  4. Non-heme: DMT-1
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21
Q

What is the key regulatory step of iron absorption?

A

Will iron be transferred from enterocyte into the blood via ferroportin

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22
Q

What is the function of transferrin?

A

Transports iron in the blood and delivers it to the liver and bone macrophages for storage.

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23
Q

Stored intracellular iron is bound by what?

Purpose of this?

A

Ferritin

Prevent free radical formation

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24
Q

What is serum iron?

A

Measurement of iron in the blood

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25
Q

What is TIBC?

A

Total-iron binding capacity which is a measure of transferrin in blood

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26
Q

What is % saturation?

What is normal

A

Percentage of transferrin molecules that are bound by iron

33%

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27
Q

What is serum ferritin?

A

Measure of iron stored in macrophages and liver

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28
Q

Iron deficiency is usually caused by what two paths?

A
  1. Dietary lack of iron

2. Blood loss

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29
Q

Most common causes of blood loss leading lack of iron? 3

A
  1. Menorrhagia
  2. Peptic ulcer disease in males
  3. Bleeding GI lesions
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30
Q

What two types of GI lesions are seen causing iron deficiency?
What population demographic specifically?

A
  1. Colon polyps/carcinoma in the Western world
  2. Hookworms in developing world

Elderly

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31
Q

What populations see menorrhagia and peptic ulcer disease?

A
  1. Menorrhagia: Females between 20 and 50

2. Peptic ulcer: Males between 20 and 50

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32
Q

Dietary deficiency is seen in what 3 manifestations?

A
  1. Infants: Human milk has very low iron
  2. Children: Poor diet or grow quickly
  3. Pregnant mothers: Need more iron for baby and herself
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33
Q

What effect does gastrectomy have on iron absorption?

A

Stomach acid normally maintains iron in Fe2+ state which absorbs fast. Without stomach acid, iron is in Fe3+ state in GI tract.

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34
Q

What are the four stages of iron deficiency?

A
  1. Storage iron depleted: Low ferritin and high TIBC
  2. Serum iron depleted: Low serum iron & percent saturated
  3. Normocytic anemia: Make fewer normal RBC’s
  4. Microcytic anemia: Make smaller and fewer RBC’s
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35
Q

What are the clinical features of iron deficiency? 6

A
  1. Anemia
  2. Koilonychia
  3. Pica
  4. Pallor
  5. Fatigue
  6. Dyspnea on exertion
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36
Q

What is Plummer Vinson syndrome? 3

How does it present?

A
  1. Severe iron deficiency anemia
  2. Atrophic glossitis
  3. Esophageal web
  4. Anemia
  5. Dysphagia
  6. Beefy-red tongue
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37
Q

Lab findings of Iron-deficiency anemia?

  1. Appearance of RBC’s
  2. RDW
  3. Ferritin
  4. TIBC
  5. Serum Iron
  6. Percent saturated
  7. Free erythrocyte protoporphyrin (FEP)
  8. Hb
  9. Hematocrit
  10. RBC count
A
  1. Small hypochromic RBC’s
  2. Increased (some normo and some micro)
  3. Decreased
  4. Increased
  5. Decreased
  6. Decreased
  7. Increased
  8. Decreased
  9. Decreased
  10. Decreased
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38
Q

What is most sensitive diagnostic of iron-deficient anemia?

What is one negative?

A

Serum ferritin

Serum ferritin elevates in pro-inflammatory states

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39
Q

Where is iron in terms of chronology of heme synthesis?

A

Last step

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40
Q

How to differ between Iron-deficiency and anemia of chronic disease?

A

Anemia of chronic disease has a low TIBC compared to ID-A.

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41
Q

How to differ between Beta-thalassemia minor and ID-A?

A

Increased Hb A2 in Beta-thalaseemia minor

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42
Q

Treatment of ID-A?

A

Supplemental iron (Ferrous sulfate)

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43
Q

Anemia of chronic disease is secondary to what types of diseases? 2

A
  1. Chronic inflammation (arthritis, infection)

2. Cancer

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44
Q

Anemia of chronic disease is most common in what population of patients?

A

Hospitalized patients

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45
Q

Chronic inflammation results in production of what?

Specifically?

A

Production of acute-phase reactants

Hepcidin

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46
Q

What is the function of hepcidin?

A

Hepcidin sequesters iron in storage sites by limiting iron transfer from macrophages to erythroid precursors AND suppressing EPO production.

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47
Q

How does A-CD appear at first?

Then what does it become?

A
  1. Normochromic and normocytic

2. Hypochromic and microcytic

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48
Q

What are the lab findings in A-CD?

  1. Ferritin
  2. TIBC
  3. Serum iron
  4. % saturation
  5. FEP
A
  1. Increased
  2. Decreased
  3. Decreased serum iron
  4. Decreased
  5. Increased
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49
Q

Treatment of A-CD? (2)

A
  1. EPO for cancer patients

2. Auto-immune disease treatment for chronic inflammed patients

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50
Q

Sideroblastic anemia is due to what?

A

Defective protoporphyrin synthesis

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51
Q

What is the first reaction in heme synthesis?

What is the co-factor?

A

Aminolevulinic acid synthetase (ALAS) converts succinyl CoA to aminolevulinic acid (ALA)

Vitamin B12

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52
Q

What is the second reaction of heme synthesis?

A

Aminolevulinic acid dehydratase (ALAD) converts ALA to porphobilinogen

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53
Q

What is the final reaction of heme synthesis?

Where is this located?

A

Ferrochelatase attaches protoporphyrin to iron to make heme

Mitochondria

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54
Q

What is the rate limiting step of heme synthesis?

A

First reaction to make ALA

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55
Q

If proto-porphyrin is deficient what happens to iron?

How does this appear?

A

Remains trapped in mitochondria

These iron-laden mitochondria form a ring around nucleus in erythroid precursors that are called ringed sideroblasts

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56
Q

What is the congenital cause of sideroblastic anemia?

A

X-linked defect in ALAS enzyme

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57
Q

What are the three acquired causes of sideroblastic anemia?

Explain each

A
  1. Alcoholism: Mitochondrial poison
  2. Lead poisoning (Inhibits ALAD and ferrochelatase)
  3. B6 deficiency: Required cofactor for ALAS and is decreased when treated with isoniazid
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58
Q

Lab findings in sideroblastic anemia?

  1. Ferritin
  2. TIBC
  3. Serum iron
  4. % saturated
  5. Cell appearance
A
  1. Increased ferritin
  2. Decreased TIBC
  3. Increased serum iron
  4. Increased % saturated
  5. Ringed sideroblasts
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59
Q

Treatment for sideroblastic anemia?

A

Pyridoxine

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60
Q

Thalassemia is an anemia due to what?

A

Decreased synthesis of globin chains of hemoglobin

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61
Q

Why is there a decrease in globin chain synthesis in thalassemia?

A

Mutations in the genes for certain globin chains

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62
Q

Mutated carriers of thalassemia have protection against what?

A

Plasmodium falciparum malaria

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63
Q

Alpha thalassemia is due to what mutation?

A

Deletion in alpha-globin gene

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64
Q

How many alpha thalassemia genes are normally present?

A

4, 2 on each chromosome

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65
Q

One gene deletion of alpha-globin results in what?

A

Asymptomatic

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66
Q

Two genes deleted of alpha-globin results in what?

A

Mild anemia with elevated RBC count.

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67
Q

A cis-deletion of alpha-globin genes has an increased risk of what?

A

Severe thalassemia in offspring

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68
Q

Cis deletion of alpha globin is seen in what population?

A

Asians

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69
Q

Trans deletion of alpha globin gene is seen in what population?

A

Africans

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70
Q

Three alpha globin genes deleted results in what?

A

Severe anemia.

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71
Q

What type of hemoglobin dominates in 3 alpha gene deletions?

A

Tetramers of Beta chains (Hemoglobin H)

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72
Q

Hemoglobin H has what effect?

A

Damages RBC membranes

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73
Q

Four genes deleted usually results in what?

A

Death of fetus (hydrops fetalis)

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74
Q

What hemoglobin dominates in 4 alpha gene deletions?

A

Tetramers of gamma chains (Hemoglobin Barts)

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75
Q

Beta thalassemia is due to what mutation?

A

Point mutation in promoter or splicing sites.

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76
Q

What population usually has Beta thalassemia? 2

A
  1. Africans

2. Mediterranean descent

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77
Q

What are the locations of alpha and beta globin genes?

A
  1. Alpha = Chromosome 16

2. Beta = Chromosome 11

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78
Q

What is Beta thalassemia minor?

A

1 normal Beta gene

1 mutated Beta gene

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79
Q

How does beta-thalassemia minor present? 6

A
  1. Asymptomatic
  2. Increased RBC count
  3. Hypochromic microcytic anemia
  4. Increased in hemoglobin A2 and HbF
  5. Decrease in hemoglobin A
  6. Target cells
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80
Q

What is beta thalassemia major?

A

Homozygous mutated Beta globin genes

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81
Q

When do symptoms of beta-thalassemia present?

A

Several months after birth when HbF begins to decrease.

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82
Q

What forms in Beta thalassemia major?

What does this result in? (2)

A

Alpha tetramers

Ineffective erythropoiesis
Extravascular hemolysis (spleen removes RBC's)
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83
Q

How does the bone marrow respond to beta thalassemia major?

What does this result in? (3)

A

Massive erythroid hyperplasia

  1. Hematopoiesis into skull (crewcut x-ray) and facial bones (chipmunk facies)
  2. Hepatosplenomegaly
  3. Risk of aplastic crisis with parvovirus B19
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84
Q

Chronic transfusions in Beta-thalassemia majorsometimes lead to what?

A

Secondary hemochromatosis (Extra iron in blood)

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85
Q

How does a smear in beta-thalassemia major appear?

A

Microcytic hypochromic RBC’s with target cells and nucleated red blood cells

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86
Q

How do hemoglobin concentrations change in beta-thalassemia major?

A

Elevated HbA2 and HbF

little or no HbA

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87
Q

What is sickle cell thalassemia the result of?

A

Coinheritance of hemoglobin S genea dn thalassemic variant of the Beta-globin gene.

88
Q

How does sickle cell’s thalassemia compare to the other Beta-thalassemias?

A

Less severe than sickle cell

89
Q

How does lead poisoning present? (LEAD)

A
  1. Lead lines on gingiva (Burton’s lines) and on metaphyses of long bones
  2. Encephalopathy and Erythrocyte basophilic stippling
  3. Abdominal colic and sideroblastic Anemia
  4. Drops of wrist and foot (Dimercaprol and eDta to treat)
90
Q

In terms of macrocytic anemia, what is the MCV?

A

Greater than 100

91
Q

Three main causes of macrocytic anemia?

A
  1. Folate deficiency
  2. B12 deficiency
  3. Orotic aciduria
92
Q

Folate and B12 are necessary for synthesis of what?

A

DNA precursors

93
Q

Folate circulates in serum as what form?

A

Methyltetrahydrofolate (methyl THF)

94
Q

Removal of methyl group from methyl-THF allows for what?

What takes the methyl group?

A

Allows folate to participate in DNA synthesis

Vitamin B12

95
Q

Vitamin B12 transfers methyl group to what?

To create what?

A

Homocysteine

Methionine

96
Q

Lack of DNA precursors has what effect on RBC’s?

A

Lack of nucleotide precursors –> RBC’s can’t divide enough –> Large RBC’s.

97
Q

Lack of DNA precursors has what effect on granulocytes?

A

Hypersegmented neutrophils

98
Q

Overall, 3 areas that see a change due to folate or B12 deficiency?

A
  1. RBC’s –> Megaloblastic anemia
  2. Granulocytes –> Hypersegmented neutrophils
  3. Megaloblastic epithelial cells
99
Q

3 other causes of macrocytic anemia without megaloblastic change? (3)

A
  1. Alcoholism
  2. Liver disease
  3. Drugs
100
Q

Dietary folate is obtained from what?

A

Green vegetables and fruit

101
Q

Dietary folate is absorbed where?

102
Q

Folate deficiency develops over what period of time?

103
Q

Causes of folate deficiency? (3)

A
  1. Poor diet: Alcoholics and elderly
  2. Increased demand: Pregnant, cancer, hemolytic anemia
  3. Folate antagonists: Methotrexate, Dilantin
104
Q

Clinical and lab findings of folate deficiency?

  1. Appearance of RBC’s and neutrophils
  2. Tongue
  3. Serum folate levels
  4. Serum homocysteine
  5. Methylmalonic acid levels
A
    1. Macrocytic RBC’s and hypersegmented neutrophils
  2. Glossitis
  3. Decreased serum folate
  4. Increased serum homocysteine
  5. Normal methylmalonic acid
105
Q

Dietary B12 enters the body how?

A
  1. Salivary gland amylase liberates B12
  2. B12 binds to R-binder (also from saliva)
  3. In the duodenum, pancreatic proteases detach vitamin B12 from R-binder.
  4. Vitamin B12 then binds intrinsic factor (from gastric parietal cells)
  5. VitaminB12-Intrinsic factor is absorbed in ileum
106
Q

Which is more common, folate or B12 deficiency?

107
Q

How long does B12 deficiency take to develop?

Why?

A

Years

Huge hepatic stores of B12

108
Q

What is most common cause of vitamin B12 deficiency?

A

Pernicious anemia

109
Q

What is pernicious anemia due to?

A

Autoimmune destruction of parietal cells of stomach –> No intrinsic factor –> No absorbed B12

110
Q

Other causes of Vitamin B12 deficiency? 5

A
  1. Pancreatic insufficiency
  2. Damage to terminal ileum (Crohn’s, tapeworm)
  3. Dietary deficiency in vegans
  4. Auto-immune gastritis
  5. Diphyllobothrium latum infections
111
Q

Lab findings in B12 deficiency?

  1. Smear appearance
  2. Methylmalonic acid
  3. Serum B12
  4. Serum homocysteine
A
  1. Macrocytic RBC’s with hypersegmented neutrophils
  2. High methylmalonic acid
  3. low B12
  4. High serum homocysteine
112
Q

Clinical findings in B12 deficiency? 2

A
  1. Glossitis

2. Subacute combined degeneration of spinal cord

113
Q

Autoimmune gastritis is associated with what other effects? (3)

A
  1. Achlorhydria (No HCl)
  2. Anti-intrinsic factor and antiparietal cell Ab’s
  3. Increased incidence of gastric carcinoma
114
Q

What test will determine if intrinsic factor alone will solve B12 deficiency?

A

Schilling Test

115
Q

Orotic aciduria is due to what?

A

Genetic mutation in enzyme that synthesizes uridine from orotic acid

116
Q

How does orotic aciduria present?

A

Megaloblastic anemia that cannot be cured by folate or B12

117
Q

Findings in orotic aciduria? (3)

A
  1. Hypersegmented neutrophils
  2. Glossitis
  3. Orotic acid in urine
118
Q

Normocytic anemia is characterized how?

A

MCV of 80 to 100

119
Q

What is normocytic anemia due to? (2)

A

increased peripheral destruction or underproduction of RBC’s

120
Q

What distinguishes whether a normocytic anemia is due to increased peripheral destruction or underproduction of RBC’s?

A

Reticulocyte count

121
Q

Reticulocytes are what?

How are they identified?

A

Young RBC’s from the bone marrow

Larger cells with bluish cytoplasm

122
Q

Normal reticulocyte count is what?

123
Q

RBC lifespan is how long?

What percentage of RBC’s are removed per day?

A

120 days

1-2%

124
Q

How can you determine if a healthy marrow is responding correctly to anemia?

A

Reticulocyte count greater than 3%

125
Q

Reticulocyte count is corrected for by doing what?

A

Multiplying reticulocyte count by Hct/45

126
Q

Corrected count >3% indicates what?

A

Good marrow resonse –> peripheral destruction

127
Q

Corrected count <3% indicates what?

A

Poor marrow response –> Underproduction

128
Q

Peripheral RBC destruction is divided into what two types?

A

Extravascular and Intravascular

129
Q

Extravascular hemolysis involves RBC destruction by what?

A

Reticuloendothelial system (macrophages of the spleen, liver, and lymph nodes)

130
Q 
In extravascular hemolysis, macrophages consume RBC's, what do the parts of the RBC become? 
Globin?
Heme?
Iron?
Protoporphyin?
A

Globin –> Amino acids
Heme –> Iron and protoporphyrin
Iron –> Recycled
Protoporphyrin –> Unconjugated bilirubin –> Binds to albumin –> delivered to liver–> Conjugated –> Excreted in bile

131
Q

Clinical and laboratory findings of extravascular hemolysis? (6)

A
  1. Anemia
  2. Splenomegaly
  3. Jaundice due to unconjugated bilirubin
  4. Bilirubin gallstones
  5. Marrow hyperplasia with RC of >3%
  6. increased LDH
132
Q

Intravascular hemolysis involves destruction of RBC’s where?

A

Within vessels

133
Q

Clinical and laboratory findings of intravascular hemolysis? (5)

A
  1. Hemoglobinemia
  2. Hemoglobinuria
  3. Hemosiderinuria
  4. Decreased serum haptoglobin
  5. Increased LDH
134
Q

What are the 3 main extravascular hemolysis normocytic anemias?

A
  1. Hereditary spherocytosis
  2. Sickle Cell anemia
  3. Hemoglobin C
135
Q

Hereditary spherocytosis is a inherited defect of what?

Specifically? (3)

A

RBC cytoskeleton-membrane tethering proteins

  1. Ankyrin
  2. Spectrin
  3. Band 3.1
136
Q

What happens to the RBC’s in hereditary spherocytosis

A

Membrane blebs are formed and lost over time –> Cells become round –> Can’t maneuver in spleen –> Splenic macrophages consume –> Anemia

137
Q

Lab findings of hereditary spherocytosis? (3)

A
  1. Spherocytes with loss of central pallor
  2. Increased RDW: Older cells smaller than newer
  3. Increased MCHC: Loss of space –> Increased Hb concentration
138
Q

Clinical findings of hereditary spherocytosis:

A
  1. Splenomegaly
  2. Jaundice with unconjugated bilirubin
  3. Increased risk for bilirubin gallstones
  4. Increased risk of parvovirus B19 infection
139
Q

What test diagnoses hereditary spherocytosis:

A

Osmotic fragility test: Spherocytes in hypotonic solution will burst

140
Q

Treatment for hereditary spherocytosis?

What does this result in?

A

Splenectomy

Anemia resolves –> Spherocytes still exist –> Howell-Jolly bodies emerge on blood smear

141
Q

Sickle cell anemia is due to what?

A

Autosomal recessive mutation in Beta chain of hemoglobin (glutamic acid [hydrophilic] to valine [hydrophobic]

142
Q

Sickle cell gene is carried by 10% of what descent?

Purpose

A

African

Protective against falciparum malaria

143
Q

Homozygous sickle cell gene results in what % of HbS?

144
Q

What does HbS do when deoxygenated?

A

Polymerizes –> Aggregates into needle like structures –> Sickle cells form

145
Q

Increased risk of sickling is in what conditions? (3)

A
  1. Hypoxemia
  2. Dehydration
  3. Acidosis
146
Q

What hemoglobin form protects against sickling?

147
Q

What treatment will increase HbF levels in sickle cell patient?

A

Hydroxyurea

148
Q

Reversible sickling results in what? (3)

A
  1. Extravascular hemolysis: Damaged RBC membranes are removed –> 3 symptoms
  2. Intravascular hemolysis: Damaged RBC’s dehydrate –> hemolysis
  3. Massive erthroid hyperplasia
149
Q

Result of amssive erythroid hyperplasia in sickle cell patients? (4)

A
  1. Hematopoiesis in skull –> Crewcut on x-ray
  2. Hematopoiesis in facial bones –> Chipmunk facies
  3. Extramedullary hematopoiesis with hepatomegaly
  4. Risk of aplastic crisis with parvovirus B19
150
Q

Irreversible sickling leads to complications of what?

A

Vaso-occlusion

151
Q

Complications of vasoocclusion include? 5

A
  1. Dactylitis
  2. Autosplenectomy
  3. Acute chest syndrome
  4. Pain crisis
  5. Renal papillary necrosis
152
Q

What is dactylitis?

A

Swollen hands and feet due to infarcts in bones

153
Q

What are consequences of autosplenectomy in sickle cell patients?

A
  1. Increased risk of infection with encapsulated organisms
  2. Increased risk of salmonella paratyphi osteomyelitis
  3. Howell-Jolly bodies on blood smear
154
Q

What is most common cause of death in sickle cell kids?

A

H. influenzae infection due to autosplenectomy

155
Q

What is most common cause of death in adult sickle cell patients?

A

Acute chest syndrome

156
Q

Sickle cell trait is the presence of what alleles?

A

One mutated and one normal Beta chain

157
Q

Sickle cell trait has what % of HbS?

A

Less than 50

158
Q

Where do people with HbS less than 50% experience sickling?

A

Renal medulla due to extreme hypoxia nad hypertonicity

159
Q

Laboratory findings in sickle cell?

A
  1. Sickle cells and target cells (Disease only)
  2. Metabisulfite screen: Causes HbS RBC’s to sickle (Both)
  3. Hb electrophoresis to confirm presence of HbS
160
Q

Full disease Sickle cell has what array of Hb’s?

A

90% HbS
8% HbF
2% HbA2
No HbA (No Beta globin chains)

161
Q

Trait sickle cell has what array of Hb’s?

A

55% HbA
43% HbS
2% HbA2

162
Q

What is hemoglobin C the result of?

Specifically?

A

Autosomal recessive mutation in Beta chain of hemoglobin

Gluatmic acid to lysine

163
Q

How does hemoglobin C present?

A

Mild anemia with extravascular hemolysis

164
Q

What is the dead giveaway in HbC lab?

A

HbC crystals in RBC’s on a smear

165
Q

What are the 5 normocytic anemias with predominant intravascular hemolysis?

A
  1. Paroxysmal nocturnal hemoglobinuria
  2. Glucose-6-Phosphate dehydrogenase
  3. Immune hemolytic anemia
  4. MIcroangiopathic hemolytic anemia
  5. malaria
166
Q

What is paroxysmal nocturnal hemoglobinuria due to?

A

Acquired defect in myeloid stem cells –> Absent glycosylphosphatidylinositol (GPI) –> Cells can be destroyed by complement

167
Q

What normally protects blood cells from complement mediated damage? 2
How?

A

DAF and MIRL

Inhibits C3 convertase

168
Q

DAF and MIRL are secured to RBC membrane by what?

169
Q

In PNH, when does intravascular hemolysis occur?

Why?

A

Usually at night during sleep

Mild respiratory acidosis during shallow breathing of sleep –> Activates complement –> RBC’s, WBC’s, and plateltes are lysed

170
Q

What are lab tests for PNH? 5

A
  1. Hemoglobinemia
  2. Hemoglobinuria
  3. Hemosiderinuria
  4. Sucrose test
  5. Acidified serum test/flow cytometry for lack of CD55/DAF on blood cells
171
Q

Main cause of death in PNH patients?

A

Thrombosis of hepatic, portal, or cerebral veins

172
Q

Why does thrombosis occur in PNH?

A

Destroyed platelets release their contents into circulation –> Induce thrombosis

173
Q

Complication of PNH? (2)

A
  1. Iron deficiency anemia

2. Acute myeloid leukemia

174
Q

Glucose-6-phosphate dehydrogenase deficiency is due to what?

A

X-linked recessive disorder resulting in reduced half life of G6PD –> Cells susceptible to oxidative stress

175
Q

How do RBC’s deal with oxidative stressors like H2O2? 3

A
  1. Glutathione neutralizes H2O2 –> Glutathione becomes oxidized
  2. G6PD generates NADPH
  3. NADPH reduces glutathione to regenerate reduced glutathione
176
Q

What are the two major variants of G6PD?

A
  1. African variant: Mildly reduced G6PD –> Mild intravascular hemolysis
  2. Mediterranean variant: Markedly reduced G6PD –> Marked intravascular hemolysis
177
Q

Carriers of G6PD are protective against what?

A

Falciparum malaria

178
Q

Oxidative stress has what effect on Hb?

A

Precipitates them into Heinz bodies

179
Q

Causes of oxidative stress? (3)

A
  1. Infection
  2. Drugs (primaquine, sulfa, dapsone)
  3. Fava beans
180
Q

Heinz bodie are removed from RBC’s by what?

A

Splenic macrophages which causes lysis

181
Q

What does G6PD deficiency present as after exposure to oxidative stress? (2)

A
  1. Hemoglobinuria

2. Back pain

182
Q

What is used to screen for G6PD?

A

Heinz preparation

183
Q

Immune hemolytic anemia (IHA) is mediated by what? 92)

A

IgG or IgM destruction of RBC’s

184
Q

IgG IHA usually involves what type of hemolysis?

A

Extravascular

185
Q

IgG in IHA does what?

A

IgG binds RBC’s in warm temp of central body (warm agglutinin) –> Membrane of antibody coated RBC consumed by splenic macrophages –> Spherocytes form

186
Q

What is IHA IgG associated with? (3)

Which one is most common?

A

SLE (most common)
CLL
Drugs: Penicillin and cephalosporins

187
Q

How do drugs cause IgG IHA? (2)

A
  1. Drug attaches to RBC membrane –> Antibody binds to drug membrane complex
  2. Drug induces production of autoantibodies that bind self antigens
188
Q

Treatment of IgG IHA (5)

A
  1. Quit the problem drug
  2. Steroids
  3. IVIG
  4. Splenectomy
189
Q

Purpose of giving IVIG to IgG IHA patient?

A

Splenic macrophages like to consume this most

190
Q

IgM mediated IHA involves what type of hemolysis?

A

Intravascular

191
Q

How does IgM cause IHA?

A

IgM binds RBC’s –> Fixes complement in cold temp of extremities (Cold agglutinin)

192
Q

IgM mediated IHA is associated with what two infections?

A
  1. Mycoplasma pneumoniae

2. Infectious mononucleosis

193
Q

What is used to diagnose IHA?

A

Coomb’s test

194
Q

Direct Coombs test confirms what?

How does it do so?

A

Presence of antibody coated RBC’s

Anti-IgG added to patient’s RBC’s –> Agglutination if RBC’s are already coated with Ab’s

195
Q

Indirect Coombs test confirms what?

How does it do so

A

Presence of antibodies in patient serum

Anti-IgG and test RBC’s are mixed with patient serum –> Agglutination occurs if serum antibodies are present

196
Q

Microangiopathic hemolytic anemia is what?

A

Intravascular hemolysis that occurs to RBC’s as they pass through ciruclation

197
Q

What are the results of microangiopathic hemolytic anemia? (2)

A
  1. Iron deficiency anemia

2. Chronic hemolysis

198
Q

What types of patients get microangiopathic hemolytic anemia?

A
  1. Microthbomi patients (TTP-HUS, DIC, HELLP)
  2. Prosthetic heart valves
  3. Aortic stenosis
199
Q

What do patients with MHA have as special cells?

A

Schistocytes

200
Q

Malaria is what?

A

Infection of RBC’s and liver with plasmodium

201
Q

What transmits malaria?

A

Female anopheles mosquito

202
Q

What happens to RBC’s in malaria?

What does this result in?

A

They rupture as part of plasmodium life cycle

  1. intravascular hemolysis
  2. cyclical fever
203
Q

P. falciparum fever frequency?

204
Q

P. vivax and p. ovale fever frequency?

A

Every other day

205
Q

What happens to spleen in malaria?

A

Consumes infected RBC’s –> Mild extravascular hemolysis –> Splenomegaly

206
Q

Anemia due to underproduction is characterized how?

A

Low reticulocyte count

207
Q

Etiologies of underproduction anemia? 3

A
  1. Causes of micro and macrocytic anemias
  2. Renal failure (decreased EPO)
  3. Damage to bone marrow precursor cells
208
Q

Anemias due to underproduction include? 3

A
  1. Parvovirus b19
  2. aplastic anemia
  3. Myelophthisic process
209
Q

Parvovirus B19 infects what cells?

What effect does this have?

A

Progenitor red cells

Halts erythropoiesis –> Significant anemia in setting of preexisting marrow stress

210
Q

What is treatment for parvovirus B19?

A

Supportive

211
Q

Aplastic anemia is what?

What does that result in? (2)

A

Damage to Hematopoietic stem cells

Pancytopenia
Low reticulocyte count

212
Q

Etiologies of aplastic anemia? (4)

A
  1. Drugs
  2. Chemicals
  3. Viruses
  4. Autoimmune damage
213
Q

Biopsy of aplastic anemia will show what?

A

Empty fatty marrow

214
Q

Treatment of aplastic anemia? (2)

A
  1. Cessation of any causative drugs

2. Supportive care of transfusions and marrow-stimulating factors

215
Q

last resort of aplastic anemia treatment?

A

Bone marrow transplant

216
Q

Myelophthisic process of anemia involves what?

A

Pathologic process (cancer) that replaces bone marrow –> Impair hematopoiesis –> Pancytopenia

Pathology (36 decks)

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