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ClinMed_I > Lecture 2a > Flashcards

Lecture 2a Flashcards

1
Q

What makes someone anemic?

A

Hgb <12 female, <13.6 male
Hct <35% female, <41% male

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2
Q

What are the causes of microcytic anemia?

A

Iron deficiency
Lead toxicity
Zinc deficiency
Thalassemia
Chronic anemia

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3
Q

What are the causes of macrocytic(megaloblastic) anemia?

A

Vitamin B12 deficiency
Folate deficiency
DNA synthesis inhibitors

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4
Q

Define intravascular hemolysis.

A

RBCs lyse within blood vessels

Note: decrease in haptoglobin, possible hemoglobinuria

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5
Q

What types of RBCs appear in intravascular hemolysis?

A

Schistocyte

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6
Q

Define extravascular hemolysis.

A

RBCs are destroyed within organs (spleen, liver)

Note: haptoglobin may not decrease

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7
Q

Why do sphereocytes form in extravascular hemolysis?

A

A little piece of RBC gets bitten off but the RBC snaps back into a normal form

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8
Q

How is iron in the cells affected between intravascular and extravascular hemolysis?

A

Intravascular: decrease in iron over time
Extravascular: iron is recovered and stored

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9
Q

When do we see spleen enlargement?

A

Extravascular hemolysis

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10
Q

When do we see red-brown urine?

A

Intravascular hemolysis

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11
Q

Why would MCV be elevated in hemolysis?

A

Because of the increase of reticulocyte count

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12
Q

Why would reticulocyte not increase?

A

Bone marrow may run out of resources to make more

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13
Q

What is a type of structural anemia?

A

Hereditary spherocytosis

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14
Q

What are types of hemoglobinopathies?

A

Thalassemias
Sickle Cell disease

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15
Q

What is a type of metabolic anemia?

A

G6PD deficiency

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16
Q

What is a type of immune-related anemia?

A

Autoimmune hemolytic anemia
Paroxysmal nocturnal hemoglobinuria
Hemolytic disease of the newborn

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17
Q

Who often inherits hereditary spherocytosis?

A

1 in 5000 northern Europeans
Autosomal dominant

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18
Q

What is the pathology behind hereditary spherocytosis?

A

Round instead of flexible biconcave disk
In small red pulp fenestrations which leads to phagocytic destruction

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19
Q

What are some symptoms of hereditary spherocytosis?

A

Mild to severe anemia
Can be undetected for years
Jaundice
Enlarged spleen
Gallstones (50% of pts)

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20
Q

What are possible treatments for hereditary spherocytosis?

A

Folic acid 1mg daily
Transfusion (EPO may reduce the need in infants)
Splenectomy only >5yo or until puberty (need antipneumococcal vaccination)

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21
Q

What tests should we order to help determine hereditary spherocytosis?

A

Blood smear
Coombs testing(should be negative)

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22
Q

What are the types of Hgbs and how prevalent they are?

A

HgbA 97-99% in RBC (2alpha, 2 beta subunits)
HgbA2 1-3% in RBC (2alpha, 2 delta)
HgbF <1% in RBC (2alpha, 2 gamma)
HgbS (2 alpha, 2 betaS)

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23
Q

What chromosomes are important for creating Hgb? What do they code for?

A

Chromosome 16: 2 copies of alpha-globing gene (4 total)
Chromosome 11: 1 copy of beta-globulin gene (2 total), as well as delta and gamma

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24
Q

What causes alpha thalassemias?

A

Gene deletions that reduce alpha-chain synthesis

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25
Q

What shows up on a Hgb electrophoresis for alpha thalassemia?

A

Equal proportions of HgbA, HgbA2, HgbF

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26
Q

What is the pathology behind alpha thalassemias?

A

Increased number of small “pale” RBCs

Excess beta chains precipitate which causes damage to RBC membranes leading to hemolysis in marrow and splenic vessels

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27
Q

Who are likely to have alpha thalassemia?

A

Southeast asian and Chinese descent
Mediterranean or African descent

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28
Q

What is the alpha thalassemia syndrome called when they have 3 normal a-globulin genes? 2,1,0?

A

3: silent carrier (alpha thalassemia minima)
2: alpha thalassemia minor
1: hemoglobin H Disease (no use for O2 transport)
0: Hydrops fetalis

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29
Q

What labs do we use to find alpha thalassemia?

A

Hgb electrophoresis (normal in minima and minor, HgbH band in HgbH disease)
Inclusion bodies (in HgbH disease)

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30
Q

What appears on a peripheral smear for alpha thalassemia trait/minor?

A

Hypochromic microcytic cells
Target cells

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31
Q

What appears on a peripheral smear for a Hgb H disease?

A

Hypochromic microcytic cells
Target cells
Poikilocytosis
Normal RBC if had transfusion

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32
Q

What treatment do you need for alpha thalassemia minima?

A

No treatment needed
Check of iron overload from transfusion

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33
Q

What treatment do you need for Hgb H disease?

A

Folic acid supplementation 1 mg/day
Splenectomy
Regular transfusion

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34
Q

What do you avoid taking when you have Hgb H disease?

A

Iron supplements
Oxidative drugs…
Sulfa drugs
Antimalarials
Isoniazid
Nitrofurantoin

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35
Q

What causes beta thalassemias?

A

Gene point mutations that cause reduced beta-chain synthesis

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36
Q

What are the types of mutations in beta thalassemias?

A

Beta+: reduced, but not absent beta-chain synthesis
Beta0: absent beta-chain synthesis

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37
Q

What shows on on a Hb electrophoresis for beta thalassemia?

A

Increased HgbA2 and HgbF

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38
Q

Who are likely to have beta Thalassemias?

A

Mediterranean descent
African and Asian descent

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39
Q

What is the pathology of beta thalassemias?

A

Excess alpha chains precipitate which damages RBC membranes causing hemolysis in marrow, spleen, and liver
Alpha chains → inclusion bodies → damaged erythroid precursors
Surviving RBCs → inclusion bodies → shortened lifespan

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40
Q

What are the types of beta thalassemias?

A

Normal
Minor
Intermedia
Major

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41
Q

What shows up on a Hgb electrophoresis for beta thalassemia?

A

Abnormal proportions of HgbA, HgbA2, HgbF

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42
Q

What exam findings would appear on a pt with beta Thalassemia?

A

Chipmunk Faces
Thinning of long bones
Pathologic fracture
Failure to thrive

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43
Q

Why are children not growing when they have beta Thalassemia?

A

They are not getting enough O2

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44
Q

What appears on a a peripheral smear for beta-thalassemia minor?

A

Hypochomic microcytic cells
Target cells

Note: same as alpha-thalassemia minor

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45
Q

What appears on a peripheral smear for beta-thalassemia intermedia?

A

Hypochomic microcytic cells
Target cells
Poikilocytes

Note: Same as Hgb H disease

46
Q

What appears on a peripheral smear for beta-thalassemia major?

A

Hypochomic microcytic cells
Target cells
Poikilocytes
Nucleated RBCs

47
Q

What is the treatment for B-minor?

A

None

48
Q

What is the treatment for B-intermedia?

A

Genetic counseling (usually)
Transfusion
Splenectomy
Avoid Iron supplements

49
Q

What is the treatment for B-major?

A

Transfusion-dependent
Splenectomy
Luspatercept - action-A trap drug
Allogenic bone marrow transplant

50
Q

What is contraindicated for Luspatercept?

A

Splenectomy
Pregnant
Breastfeeding

51
Q

What is the MOA for Luspaterecept?

A

Promotes production of RBCs by interfering with TGF-beta signaling

52
Q

What is the dosing for Luspatercept?

A

SC injection every 3 weeks

53
Q

What are the SE to Luspatercept?

A

Decreased Creatine clearance
Increase LFTs (liver function test)
MSK pain
GI upset
HTN
Extramedullary hematopoietic masses
Thromboembolisms

54
Q

Why do we worry about iron overload?

A

The spleen destroyed RBCs but the heme is still there so careful when taking iron supplements

55
Q

What is the cause of SCA?

A

Autosomal recessive inherited disease affecting betaS gene (affects HgbS)
Heterozygous: sickle cell trait (asymptomatic)
Homozygous: sickle cell anemia

56
Q

Who are most likely to get SCA?

A

African Americans 1/400 in US
Mediterranean, Latin, Native American Descent

57
Q

What is the pathology behind SCA?

A

Unstable HbS causes “sickeled cells” causing vaso-occlusive episodes(block blood flow)

58
Q

Why do sickle cells cause vasoocclusion?

A

They are inflexible and stick to endothelium

59
Q

What can trigger vast-occlusive episodes in SCA?

A

Hypoxemia
Acidosis
Infection
Excessive exercise
Abrupt temp changes
Anxiety/stress

60
Q

What shows up on a Hgb electrophoresis in SCA?

A

Abnormal proportions of HgbA, HgbA2, HgbF
Presence of HgbS band

61
Q

What appears on a peripheral smear in SCA?

A

Target cells
Sickled RBCs
Howell-Jolly inclusion body

62
Q

When do S/S appear in SCA? What are they?

A

6 month
Jaundice, pallor
Pain
Retinopathy
Splenomegaly
Hepatomegaly
Gallstones
Cardiomegaly
Hyperdynamic precordium

63
Q

What are the symptoms of SCA on the extremities?

A

Non healing wound
Dactylitis(swelling and inflammation of digits)

64
Q

Where are the locations of sickle cell crisis(ischemic injury)?

A

Abdomen, bones, joints, soft tissue
Less than 18months: hands/feet
Childhood/teens: bones of arms and legs
Adults: vertebrae

65
Q

What are some complications of sickle cell?

A

Bone necrosis
Cerebral atropy
CKD
Cardiac enlargement/heart failure
HTN
Hyposplenism
Retinopathy
Hepatomegaly, hepatic failure
Sickle cell crisis

66
Q

What makes sickle cell anemia so bad compared to other anemias?

A

To blocks blood vessels and cuts off blood flow to tissues

67
Q

What can we give to support SCA?

A

Folic Acid
Vacines (pnuemococcal, covid, influenza)
Ace inhibitors (protect from proteinuria)
Omega-3-fatty acids (reduce crisis)
Transfusion
Pain management
Antibiotics

68
Q

Why do we give prophylactic antibiotics to SCA pts?

A

So they dont get sick

69
Q

What are the disease-modifying medications for SCA?

A

Hydroxyurea
Crizanlizumab
L-glutamine

70
Q

How does hydroxyurea work?

A

Increase HgbF levels
Reduces vasoocclusive episodes

71
Q

What is the major SE of hydroxyruea?

A

Bone marrow suppression
Vasculitis skin ulcer
Increase cancer risk
Teratogenic (don’t use if pregnant)

72
Q

What medications do we give to SCA if they cannot tolerate hydroxyurea?

A

Crizanlizumab
L-glutamine

73
Q

How does crizanlizumab work?

A

Monoclonal antibody against P-selectin proteins on endothelial cells and platelets
Reduces vasoocclusive episodes by reducing interactions between RBCs and endothelium

74
Q

What are the SE and dosage of crizanlizumab?

A

Some joint pain
IV every 2-4 weeks, expensive

75
Q

How does L-glutamine work?

A

Decreases vasoocclusive episodes by reducing oxidative stress that promotes sickling

76
Q

What are the SE and dosage for L-glutamine?

A

Joint pain
Powder, mixed with food and water, expensive

77
Q

What are the treatments for SCA?

A

HOP
Hydration
Oxygenation
Pain control
Transfusion
Allogenic hematopoietic stem cell transplant

78
Q

What is a splenic sequestration crisis?

A

Disproportionate amount of blood being sequestered in spleen leading to shock
Enlarges spleen and and Hgb drops >2 baseline
10-15% mortality rate

79
Q

What causes G6PD deficiency?

A

X-linked recessive genetic defect

80
Q

Who are likely to be G6PD deficient?

A

African-American males
Asian and Mediterranean descent
Rarely seen in females

81
Q

What is the pathology behind G6PD deficiency?

A

Oxidative stress → Hb denatures, forms precipitate (Heinz bodies)
Heinz bodies damage RBC membrane → destruction by spleen
Cells can also rupture spontaneously (intravascular)

82
Q

What are some S/S of G6PD deficiency?

A

Usually asymptomatic
Episodic hemolytic anemia
Jaundice in newborns

83
Q

What symptoms appear in an acute episode in G6PD deficiency?

A

Malaise
Weakness
Abdominal
Lumbar pain
Jaundice
Dark urine

84
Q

What shows on a peripheral smear for a G6PD deficiency?

A

Bite cells
Blister cells
Polychromataphils/Reticulocytes
Heinz bodies if special stained

85
Q

What are some preventive measures for G6PD?

A

Avoiding oxidant drugs, trigger foods
Genetic counseling

86
Q

What are some therapeutic measures for G6PD?

A

Removing offending agent
Folic acid supplementation
Transfusion

87
Q

Do we monitor for iron overload in G6PD deficiency?

A

No, they hardly need transfusions

88
Q

What can cause autoimmune hemolytic anemia?

A

50% idiopathic
Lupus
Lymphocytic leukemia
Lymphoma
Drugs: Antibiotics, methyldopa, levodopa, NSAIDs

89
Q

What are some symptoms that present in an autoimmune hemolytic anemia pt?

A

Abrupt, rapid onset with fatigue, jaundice, splenomegaly
10% mortality

90
Q

What types of RBCs are formed in autoimmune hemolytic anemia?

A

Spherocytes

91
Q

What is the pathology for autoimmune hemolytic anemia?

A

Tags RBCs for destruction by immune system
Complements can also tag to be destroyed by Kupffer cells in liver
IGM help facilitate MAC

92
Q

What are the two types of autoimmune hemolytic anemia?

A

Warm: happens at normal body temp
Cold: happens at colder temp

93
Q

What is the difference between a direct and indirect Coombs test?

A

Direct: a pts own blood is tested with reagent
Indirect: we take pts serum(has Ig’s) and combine with donors blood and then add reagent

94
Q

What diagnostic test do we give to determine if a pt has autoimmune hemolytic anemia?

A

Antiglobulin (Coombs) test

95
Q

What shows up on a peripheral smear in autoimmune hemolytic anemia?

A

Marked microsphereocytes
Polychromataphils/reticulocytes

96
Q

What are the possible treatments for autoimmune hemolytic anemia?

A

Immunosuppressants (Prednisone, rituximab)
Treat underlying diseases
Transfusion

97
Q

How does hemolytic disease of the newborn occur?

A

Mother develops antibodies(IgG) and tags the fetal RBCs which causes the own baby’s immune system to attack and hemolysis their own RBCS

98
Q

Who are likely to cause hemolytic disease for the newborn?

A

Rh- mother
Rh+ father/fetus

99
Q

What are some symptoms for the newborn with hemolytic disease?

A

Jaundice
Anemia
Hepatomegaly
Splenomegaly
Edema
HF

100
Q

Which Coombs test is positive for hemolytic disease of the newborn?

A

Direct Coombs is positive for the newborn
Indirect Coombs is positive for mother

101
Q

What are some treatments for hemolytic disease of newborn?

A

Before birth: fetal transfusion, early labor, maternal plasma exchange(reduces Ig levels)
After birth: transfusion, supportive care
Preventative care: RhoGAM

102
Q

Who are likely to have Paroxysmal Nocturnal Hemoglobinuria?

A

Young adults
Equal in any gender at any age

103
Q

What causes paroxysmal nocturnal hemoglobinuria?

A

Acquired genetic defect that causes RBCs lysis
CD55 and CD59 (regulates MAC)

104
Q

What substance helps get rid of Hgb?

A

NO

105
Q

What are some symptoms of low NO?

A

Esophageal spasms
Erectile dysfunction
Renal Damage
Thrombosis

106
Q

Why does paroxysmal nocturnal hemoglobinuria tend to occur at night?

A

Blood pH drops at night which helps in the process of RBC hemolysis

107
Q

What are some symptoms of of Paroxysmal Nocturnal Hemoglobinuria?

A

Thrombosis on veins of mesentery, liver, skin and CNS
Anemia

108
Q

What diagnostic tests do we give to check for paroxysmal nocturnal hemoglobinuria? Which is gold standard?

A

Urine hemosiderin
Flow cytometry (gold standard)

109
Q

What treatments are there for paroxysmal nocturnal hemoglobinuria?

A

Allogenic hematopoietic stem cell transplant
Eculizumab
Transfusion
Iron replacement therapy
Corticosteroids (short-term)

110
Q

What is the difference between the acute and chronic blood loss?

A

Acute is direct loss of RBC
Chronic is depletion of iron stores

111
Q

What are some symptoms of blood loss?

A

Hypovolemia: can lead to hypoperfusion

111
Q

What are some symptoms of blood loss?

A

Hypovolemia: can lead to hypoperfusion

ClinMed_I (15 decks)

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