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Surgery EOR > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

1
Q

What makes someone anemic?

A

Hbg below 11
Hct below 35

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2
Q

3 major causes of anemia

A

Decreased production of RBC
Increased destruction of RBC
Blood loss

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3
Q

Intravascular anemia

A

RBCs lyse within the blood vessels

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4
Q

Extravascular anemia

A

RBCs are destroyed within organs (spleen, liver)

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5
Q

Iron levels in intravascular anemia

A

Decrease in iron over time

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6
Q

___ are formed in intravascular anemia

A

Schistocytes

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7
Q

What are common things to see in intravascular anemia?

A

Hemoglobinuria
Decrease in haptoglobin

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8
Q

Iron levels in extravascular anemia

A

Iron is stored and recovered

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9
Q

How much Hgb is released into circulation in intravascular anemia?

A

Large amounts

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10
Q

How much Hgb is released into circulation in extravascular anemia?

A

Minimal

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11
Q

___ are formed in extravascular anemia

A

Spherocytes

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12
Q

Why would mean cell volume be increased in hemolysis?

A

Increased reticulocytes

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13
Q

Haptoglobin levels after inflammation

A

Increases and can mask changes in haptoglobin

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14
Q

Cause of hereditary spherocytosis

A

Inherited genetic effect that causes abnormal formation of proteins in RBC membrane

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15
Q

Pathology of hereditary spherocytosis

A

Abnormally shaped RBCs that are round instead of flexible disks

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16
Q

What does a lack of flexibility of the RBCs cause?

A

Causes them to get trapped in the spleen

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17
Q

Presentation of hereditary spherocytosis

A

Varying degrees of anemia

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18
Q

What may cause hereditary spherocytosis to present?

A

Infection

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19
Q

Treatment for hereditary spherocytosis?

A

Folic acid supplementation
Blood transfusions
Splenectomy

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20
Q

Why do we want to delay a splenectomy until after the patient is 5 years old?

A

Want to give the immune system time to mature and develop

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21
Q

Hemoglobin A

A

Normal adult Hgb
97-99%
aabb

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22
Q

Hemoglobin A2

A

1-3%
aadd

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23
Q

Hemoglobin F

A

Fetal hemoglobin
Less than 1%
aagg

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24
Q

What chromosome holds 2 copies of the alpha gene?

A

16

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25
Q

What chromosome holds 1 copy of the beta gene, delta gene, and gamma gene?

A

11

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26
Q

Cause of alpha thalassemia

A

Gene deletion causing reduced alpha chain synthesis

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27
Q

The ___ mutated the gene, the worse the anemia

A

More

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28
Q

What proportions would you see on a Hgb electrophoresis in alpha thalassemia?

A

Equal proportions of A, A2, and F

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29
Q

Pathology of alpha thalassemia

A

Increased number of small, pale RBCs

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30
Q

Why do you have excess B chains in alpha thalassemia?

A

The beta chains are being produced normally but the alpha chains are not being made to match with them

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31
Q

What will excess beta chains cause?

A

Damage to RBC membranes causing hemolysis in marrow and splenic vessels

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32
Q

Most common alpha thalassemia patients

A

Southeast Asia and Chinese

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33
Q

Silent carrier (alpha thalassemia minima)

A

3 normal and 1 abnormal genes

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34
Q

Alpha thalassemia minor (trait)

A

2 normal and 2 abnormal genes

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35
Q

Hemoglobin H disease

A

1 normal and 3 abnormal genes
Results in little to no use for O2 transport

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36
Q

Hydrops fetalis

A

All 4 genes are abnormal
Results in death of fetus

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37
Q

What type of anemia is alpha thalassemia?

A

Microcytic, hypochromic

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38
Q

Inclusion bodies

A

When beta chains precipitate and cause damage to the cells

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39
Q

Peripheral smear of alpha thalassemia

A

Microcytic, hypochromic
Target cells

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40
Q

Treatment for hemoglobin H disease

A

Folic acid supplementation

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41
Q

Why should you monitor iron levels in hemoglobin H disease?

A

It’s extravascular

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42
Q

Cause of beta thalassemia

A

Gene point mutations causing reduces beta chain synthesis

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43
Q

Pathology of beta thalassemia

A

Increased number of small, pale RBCs

44
Q

Why do we see altered proportions of Hb subtypes in the Hb?

A

They don’t need beta chains for the other types so it will fall back on those other types

45
Q

Demographics of beta thalassemia patients

A

Mediterranean

46
Q

Beta thalassemia minor

A

1 normal and 1 abnormal gene

47
Q

Beta thalassemia intermedia (mild)

A

Requires occasional transfusions
Has mostly HgF

48
Q

Beta thalassemia major

A

Has all mutated genes
Has mostly HgF
Is dependent on regular transfusions

49
Q

Why do we see failure to thrive in beta thalassemia?

A

Decreased O2

50
Q

What are exam findings of beta thalassemia?

A

Chipmunk faces

51
Q

Why do you see nucleated RBC in beta thalassemia major?

A

Because bone marrow is pushing out RBC so often and fast

52
Q

Only definitive treatment for beta thalassemia major

A

Allogenic bone marrow transplant

53
Q

Cause of sickle cell anemia

A

Genetic disorder that causes abnormal substitution of an amino acid in the beta chain

54
Q

Hemoglobin S

A

Present in sickle cell anemia

55
Q

Demographics of sickle cell anemia patients

A

African Americans

56
Q

Pathology of sickle cell anemia

A

Abnormally shaped RBCs causes vaso-occlusive episodes

57
Q

Vaso-occlusion

A

Cells stick to the endothelium causing ischemia, pain, and end-organ damage

58
Q

Triggers of sickle cell anemia flare ups

A

Hypoxemia
Infection
Exercise
Stress
Abrupt temperature changes

59
Q

Sickle cell trait vs. sickle cell anemia

A

Sickle cell trait: only 1 mutation and doesn’t show symptoms
Sickle cell anemia: 2 mutations and clinically present

60
Q

Why is WBC count elevated in sickle cell anemia?

A

Increased inflammation from vaso-occlusive episodes

61
Q

Key sign on peripheral smear that patient has sickle cell anemia

A

Howell-jolly inclusion body

62
Q

Sickle cell crisis

A

Condition in sickle cell anemia in which the sickled cells interfere with oxygen transport, obstruct capillary blood flow, and cause fever and severe pain in the joints and abdomen

63
Q

What would you see in the extremities of a patient with sickle cell anemia

A

Non-healing wounds and dactylitis

64
Q

What would you see in the retinal exam of a patient with sickle cell anemia?

A

Hemorrhages and cotton wool spots

65
Q

What would you see on an abdominal exam of a patient with sickle cell anemia?

A

Hepatomegaly and splenomegaly

66
Q

Treatment of sickle cell anemia

A

Avoidance of triggers
Folic acid supplementation
Stem cell transplant

67
Q

Cause of G6PD deficiency

A

X-linked recessive genetic trait

68
Q

Pathology G6PD deficiency

A

RBCs especially vulnerable to oxidative stress leading to formation of heinz bodies

69
Q

Demographics of patients with G6PD deficiency

A

African American males

70
Q

Why is G6PD deficiency rarely seen in females?

A

Because it’s X-linked

71
Q

Presentation of G6PD deficiency

A

Episodic hemolytic anemia

72
Q

3 main triggers of G6PD deficiency anemia

A

Infection
Food
Medications (3 As: antibiotics, Azo, aspirin)

73
Q

What type of cells would you see on a peripheral smear of someone with a G6PD deficiency?

A

Bite cells
Blister cells
Reticulocytes
Heinz bodies

74
Q

G6PD deficiency treatment

A

Avoid triggers
Folic acid

75
Q

Why do we not need to monitor iron levels in G6PD deficiency anemia?

A

It’s episodic

76
Q

Cause of autoimmune hemolytic anemia

A

Antibody forms against surface antigens of RBC

77
Q

Pathology of autoimmune hemolytic anemia

A

RBCs tagged for destruction by the immune system

78
Q

In autoimmune hemolytic anemia, splenic macrophages remove portions of RBC membrane leads to ___

A

Spherocyte formation

79
Q

Presentation of autoimmune hemolytic anemia

A

Abrupt, rapid, potentially life-threatening anemia

80
Q

Diagnostic test for autoimmune hemolytic anemia

A

Coombs test

81
Q

Direct Coombs test

A

Reagent is mixed with patient’s RBC

82
Q

Indirect Coombs test

A

Serum is mixed with RBCs and then reagent is added

83
Q

Treatment for autoimmune hemolytic anemia

A

Immunosuppression
Transfusions

84
Q

Why is immunosuppression key in autoimmune hemolytic anemia?

A

Problem is immune system, not RBC, so unless we suppress the immune system, then the cells will continue to get destroyed, no matter how much RBC are supplemented

85
Q

Why do you need to type/cross match transfusions for autoimmune hemolytic anemia treatment?

A

Cells are already getting destroyed so it won’t matter much if the new ones do too. We are just trying to get as many RBCs as possible

86
Q

Erythroblastosis fetalis

A

Hemolytic disease of the newborn

87
Q

Cause of hemolytic disease of the newborn

A

Maternal IgG to antigens on surface of fetal RBCs

88
Q

Pathology of hemolytic disease of the newborn

A

Maternal IgGs to fetal RBCs cross placenta to fetal circulation and bind to fetal RBCs

89
Q

Classic case of hemolytic disease of the newborn

A

Rh- mother and Rh+ father have an Rh+ baby

90
Q

Presentation of hemolytic disease of the newborn

A

Jaundice
Anemia at birth

91
Q

Why is the indirect Coombs test on the fetus negative and the mother positive?

A

Baby: because the baby is not the one making the antibodies
Mother: the mother is the one making antibodies

92
Q

Why is the direct Coombs test positive on the baby and negative on the mother?

A

Mother: because the antibodies are not attacking her own RBCs
Baby: the baby’s RBCs are coated in the mother’s antibodies

93
Q

Hemolytic disease of the newborn treatment

A

Before birth: transfusion and early delivery
After birth transfusion

94
Q

RhoGAM

A

Used to prevent an immune response to Rh positive blood in people with an Rh negative blood type

95
Q

Cause of paroxysmal nocturnal hemoglobinuria

A

Acquired genetic defect leading to lysis of RBCs

96
Q

Pathology of paroxysmal nocturnal hemoglobinuria

A

RBCs are vulnerable to lysis by complement

97
Q

Who is paroxysmal nocturnal hemoglobinuria most common in?

A

Young adults

98
Q

Presentation of paroxysmal nocturnal hemoglobinuria

A

Hemoglobinuria present first thing in the morning
Improves throughout the day

99
Q

Why does hemoglobinuria only occur first thing in the morning in paroxysmal nocturnal hemoglobinuria patients?

A

Drop in blood pH overnight facilitates hemolysis of RBC

100
Q

Do you need to monitor iron in paroxysmal nocturnal hemoglobinuria?

A

Yes, it is intravascular so it may get low

101
Q

Gold standard diagnostic test for paroxysmal nocturnal hemoglobinuria

A

Flow cytometry

102
Q

Treatment for mild paroxysmal nocturnal hemoglobinuria

A

No treatment needed

103
Q

Treatment for severe or aplastic paroxysmal nocturnal hemoglobinuria

A

Stem cell transplant

104
Q

Treatment for hemolysis or thrombosis paroxysmal nocturnal hemoglobinuria

A

Monoclonal antibodies

105
Q

Pathway of anemia due to blood loss

A
  1. Hypovolemia
  2. Fluids are replaced and patient is now anemic
  3. Recovery - bone marrow attempts to replace lost RBCs

Surgery EOR (6 decks)

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