Anemia Flashcards

1
Q

RBC Indices of Anemias

A

1) Microcytic/Hypochromic (MCV <80 fl, MCHC <31 g/dl)
2) Normocytic/Normochromic (MCV = 80-100 fl, MCHC = 31-37 g/dl)
3) Macrocytic/Normochromic (MCV >100 fl, MCHC 31-37 g/dl)

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2
Q

Anemia Based on Reticulocyte Count and DAT

A
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3
Q

Evaluation of Microcytic Anemia = MCV <80 fl

A
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4
Q

Evaluation of Macrocytic Anema - MCV >100 fl

A
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5
Q

Evaluation of Normocytic Anemia

A
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6
Q

Anemias Classified as Microcytic/Hypochromic

A

1) Iron deficiency
2) Sideroblastic Anemia
3) Thalassemia

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7
Q

Macrocytic/Normochromic

A

1) Megaloblastic Anemia
2) Liver disease
3) Any hemolytic diseases can have increased MCV but lots of polys

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8
Q

Normocytic/Normochromic

A

1) Blood loss, endocrine disease
2) Renal disease
3) Aplastic anemia
3) RBC Aplasia
4) Myelophthisic anemia

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9
Q

Non-immune Extrinsic Anemia

A

1) Infectious Agents: Malaria, Babesia, Bartonella, Clostridum
2) Venoms
3) Chemicals and Drugs
4) Abnormal lipid issues causing membrane abnormalities: Spur Cell Anemia, Abetalipoproteinemia, Lecithin-cholesterol acyl transferase (LCAT)

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10
Q

Non-Immune Physical Anemia

A

1) Microangiopathic Hemolytic Anemia
2) HELLP syndrome
3) Extreme exercise, hypertension, hypersplenism
4) Hemolytic Uremic Syndrome

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11
Q

Immune Hemolytic Extrinsic Anemia

A

1) Alloimmune: HDN, Drug induced, Transfusion, Warm autoimmune hemolytic anemias, Cold autoimmune, Raynauds, PCH

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12
Q

Non-Immune Hemolytic Intrinsic, Membrane Anemia

A

Decreased stability, loss of membrane, variation in shape, decreased flexibility, altered permeability
1) Hereditary Spherocytosis
2) Hereditary Elliptocytosis
3) Hereditary Stomatocytosis
4) Hereditary Pyropoikilocytosis
5) PNH

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13
Q

Non-Immune Intrinsic, Enzyme Anemia

A

1) Glucose-6-phosphate Dehydrogenase deficiency (G6PD)
2) Pyruvate Kinase Deficiency

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14
Q

Non-immune Intrinsic, Hemoglobinopathy Anemias

A

Gene mutation that leads to abnormal function
1) Sickle Cell Anemia
2) Sickle Cell Trait
3) Hgb C Disease
4) Hgb C Trait
5) Hgb SC
6) Hgb D disease
7) Hgb E
8) Unstable Hgbs

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15
Q

Non-Immune Intrinsic
Thalassemias

A

Gene mutation that leads to decreased synthesis of globin chains
1) Beta Thal major
2) Beta Thal minor
3) Beta Thal + Hgb S
4) Alpha Thal major
5) Alpha Thal Trait
Silent carriers
6) Hgb H disease
7) Hydrops fetalis
8) Hereditary persistence of Fetal Hgb

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16
Q

Porphyrias - Not Hemolytic Intrinsic

A

Problem with any 8 enzymes needed to change porphyrins into heme - leads to build up
1) Acute porphyrias - affects nervous system
2) Cutaneous porphyrias - mainly affect the skin

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17
Q

G6PD (Glucose-6-Phosphate Dehydrogenase)

A

Normocytic/Normochromic
Inherited x-linked
Deficiency of G6PD (buildup of oxidants)
Hgb converted to methgb (Heinz bodies)
Increased retic, bilirubin, hgburia, hgemia, “bite” cells
Treatment - avoid oxidant drugs

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18
Q

Pyruvate Kinase Deficiency

A

Normocytic/Normochromic
AR (autosomal recessive)
Alteration RBC membrane = K+ loss
Increased 2,3 DPG
Jaundice
Increased retic, decreased haptoglobin, Burr cells
Treatment - none required, transfuse if needed

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19
Q

Hereditary Spherocytosis

A

Normocytic/Normochromic
AD (autosomal dominate)
Deficiency in spectrin = unstable membrane
Membrane loss = decreased S:V ratio
Increased permeability to Na+
Hgb = normal to decreased

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20
Q

Hereditary Elliptocytosis

A

Normocytic/Normochromic
AD inherited
Increased permeability to Na+
+/- Hgb
Mildly increased retic
Elliptocytes >25%
Treatment: None, Splenectomy

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21
Q

Hereditary Stomatocytosis

A

Normocytic/Normochromic
AD inheritance
Less flexible, osmotic lysis in spleen
Increased permeability to Na+ & K+
Hgb 8-10 g/dl - mild to moderate
Increased retic, bilirubin
Stomatocytes (10-50%)
Treatment: None, Splenectomy

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22
Q

Hereditary Pyropoikilocytosis (HPP)

A

Rare AR disease
Severe subtype of Hemolytic Enzyme
Primarily African descent
Thermal stability (cells fragment at 45-46 C)
Disintegrate when incubated at 37 C for >6 hrs
2 Defects- one from each parent: Deficiency of alpha-spectrin, mutant spectrin
Striking poikilocytes (budding, fragments, microspherocytes, elliptocytes, triangulocytes, bizarre forms)
MCV (25-55 fL due to RBC fragments)
Osmotic fragility - thermal sensitivity

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23
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH)

A

Acquired: Idiopathic, Post bone marrow dmg
Abnormal clone of stem cells
Mutation in PIG-A gene causing deficiency in GPI anchor proteins
Abnormal sensitivity to complement lysis
Sleep associated hemolysis, Abdominal/low back pain, Thrombosis, Infection, Bone marrow hypoplasia
Hgb (8-10 mg/dl)
Increased retic, nRBCs, hemosiderinuria,
Testing: Sugar Water test, Acid Serum test (Ham’s), Immunophenotyping

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24
Q

Extrinsic Defects

A

1) Chemicals & Drugs
2) Oxidant Drugs, Gasses, Lead
3) Venoms
4) Infectious Agents: Babesiosis, Bartonella, Clostridium, Malaria
Identification: Thin and Thick smears

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25
Q

Abnormal Plasma Lipids

A

Normocytic, Normochromic
Spur Cell Anemia
Severe Liver disease
Increased cholesterol RBC membrane: Decreased deformability
Hgb 5-10 g/dl
Increased retic, bilirubin, abnormal liver function tests
Target Cells
Leptocytes
Spur Cell
Acanthocyte

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26
Q

Abnormal Plasma Lipids Abetalipoproteinemia

A

Normocytic, Normochromic
AR inheritance
Absence of Beta-lipoprotein = abnormal membrane
+/- Decreased Hgb
Acanthocytes

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27
Q

LCAT (Lecithin-Cholesterol Acyl Transferase) deficiency

A

Low HDL - serum lipoprotein A1 and A2
Elevated levels of APO E
Numerous macrocodocytes (Target Cells)

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28
Q

Physical Injury

A

Usually Normocytic/Normochromic
Intravascular hemolysis
Fragments
Helmet cells

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29
Q

Microangiopathic Hemolytic Anemia

A

Lesion in circulation, mechanical injury, hypertension
Artificial Heart Valves
Thrombotic Thrombocytopenic Pupura (TTP)
Disseminated Intravascular Coagulation (DIC)
Burns
Fragments

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30
Q

Hemolysis, Elevated Liver Enzymes, Low Platelets (HELLP) Syndrome

A

Obstetric patients
Similar to TTP and HUS

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31
Q

Malignant Hypertension

A

Elevated Blood Pressure

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32
Q

March Hemoglobinuria

A

Post extreme exercise
Usually no fragments

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33
Q

Hypersplenism

A

Splenic sequestration of RBCs

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34
Q

Hemolytic Uremic Syndrome (HUS)

A

Normocytic/Normochromic
Usually occurs in children
In adults - associated with bacterial infection, SLE, cancer
Acute Renal Failure (clots in kidney)
Decreased platelets
Increased retics, Decreased haptoglobin, hemoglobinemia, Hemoglobinuria
Increased BUN & Creatinine
Casts in urine

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35
Q

Immune Hemolytic Anemia - Alloimmune

A

Transfusion Reaction
Hemolytic Disease of the Newborn - mother’s antibodies causing issues

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36
Q

Immune Hemolytic Anemia - Drug Induced

A

Drug Protein Complex
Antibodies

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37
Q

Kleihauer-Betke Stain for Fetal RBCs

A

Maternal Blood sample examined for presence of fetal RBCs
Fetal RBCs may cause/increase maternal antibody production that could affect pregnancy/future pregnancies

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38
Q

Warm, Autoimmune Hemolytic Anemia (WAIHA)

A

Normocytic/Normochromic
IgG Antibody
Extravascular hemolysis
Idiopathic: associated with SLE, CLL, viral illness
Increased retics
Spherocytes

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39
Q

Cold, Autoimmune Hemolytic Anemia

A

Cold Agglutinin disease
IgM directed against I antigen
Extravascular hemolysis or Complement lysis
Idiopathic: associated with mycoplasma, MPD, viral illness
Raynaud’s Phenomenon
Anemia
Increased MCV
Corrects with higher temperature

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40
Q

Paroxysmal Cold Hemoglobinuria (PCH)

A

Biphasic IgG Antibody: Donath Landsteiner Antibody
Reacts with RBC @ <15 C; hemolysis at body temperature
Idiopathic: associated with viral infection, tertiary syphilis
Massive hemolysis with exposure to cold

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41
Q

Hemoglobinopathies

A

Abnormality in globin part of hemoglobin molecule
Amino Acid substitution in globin chain
Affects solubility, function stability of Hgb
Associated with chronic hemolytic anemia
Usually Inherited

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42
Q

Sickle Cell (SS) Comments

A

Most Common Hemoglobinopathy
Hgb S precipitates with deoxygenation
Organ/tissue infarction: Necrosis, Autosplenectomy
Hgb (6-10 g/dl)
MCV may be increased

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43
Q

Sickle Cell (homozygous) Amino Acid substitution

A

Valine for Glutamic Acid on #6 Beta chain (Hgb S)

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44
Q

Hemoglobin C Disease (CC) Comments

A

3rd Most Common
Decreased Hgb solubility
Splenomegaly, Joint Pain
Hgb 8-12 g/dl
Increased retics

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45
Q

Hemoglobin C Disease Amino Acid substitute

A

Lysine for Glutamic Acid at #6 Beta chain

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46
Q

Hemoglobin S-C Disease Comments

A

Normocytic/Normochromic
Hgb 10-14 g/dl

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47
Q

Hemoglobin D Disease - Hemoglobinopathy

A

Inherited (homozygous and heterozygous)
Several variants - Punjab, Los Angeles
Target Cells
Hemoglobin Electrophoresis
- Increased Hgb D

48
Q

Hemoglobin D Disease Amino Acid

A

Glutamine for Glutamic Acid at #121

49
Q

Hemoglobin E Disease - Hemoglobinopathy

A

Inherited (homozygous and heterozygous)
Slightly unstable Hgb with oxidant stress
Target Cells
Hemoglobin Electrophoresis
- Increased Hgb E

50
Q

Hemoglobin E Disease Amino Acid

A

Lysine for Glutamic Acid at #26

51
Q

Unstable Hemoglobins

A

Normocytic/Normochromic
Amino acid mutations that alter Hgb stability
Inherited, spontaneous, post drug or infection
Precipitation of Hgb as Heinz bodies
Rigid RBCs = hemolysis
Extravascular hemolytic anemia
Jaundice
Splenomegaly
Cyanosis - formation of sulfhemoglobin and methemoglobin
Decreased Hgb
Fragments - “bite” cells
Abnormal heat instability test
Heinz bodies

52
Q

Thalassemia General Characteristics

A

Inherited (AD)
Mediterranean descent
Chronic hemolysis with bone changes
Often confused with Fe deficiency
Genetic mutations that decreases the synthesis of globin chains

53
Q

Beta-Thalassemia Major

A

Microcytic/Hypochromic
Both beta genes affected
- B0/B0, B+/B0, B+/B+
Clinical: severe anemia, hemolysis, cardiac failure, splenomegaly, bone marrow hyperplasia
Decreased Hgb (severe anemia)
Increased retic
Serum Fe and Ferritin are normal to increased

54
Q

Beta-Thalassemia Minor

A

Inherited (heterozygous)
B+/B, B0/B
Asymptomatic unless stressed
Lots of RBC morphology for mild anemia
Hgb Electrophoresis
- Increased Hgb A2 5%
- Normal to Increased Hgb F

55
Q

Beta-Thalassemia/Hemoglobin S

A

Microcytic Hypochromic
Inherited (heterozygous)
- B+/S (few symptoms)
- B0/S (severe anemia)
Poikilocytosis
Target Cells
Increased retic

56
Q

Alpha Thalassemia General Characteristics

A

Blacks, Indians, Chinese, S.E. Asians, Middle East
Decreased synthesis of alpha chains
4 alpha genes
Mutation causing deletion of abnormality of 1 or more alpha genes

57
Q

Silent Carrier - Alpha Thalassemia

A

Normocytic/Normochromic
3 normal alpha genes and 1 abnormal
Asymptomatic

58
Q

Alpha Thalassemia Trait (Minor)

A

Mild microcytic/hypochromic
2 normal alpha genes and 2 abnormal
Excess Beta chains form tetrads = B4
Beta tetrads = Hgb H
Hgb H is unstable = Heinz bodies
Hgb H has high affinity for O2

59
Q

Alpha Thalassemias

A

Microcytic/Hypochromic
Hgb H disease - 1 normal alpha gene, 3 abnormal alpha genes
Increased retic
Excess Beta chains

60
Q

Hydrops fetalis Alpha Thalassemia (Major)

A

Hypochromic anemia
No normal alpha genes
Excess gamma chains in fetus
Gamma4 = Hgb Barts
Hgb Barts has high O2 affinity
Hypoxia - stillborn or die within hrs of birth
Increased retic
High RDW
nRBCs
Poikilocytosis

61
Q

Hereditary Persistence of Fetal Hemoglobin

A

Microcytic/Hypochromic
Inherited
No production of Delta or Beta chains
Production of Gamma chains persists throughout life
Hgb F
Increased Hgb due to increased O2 affinity of Hgb F
High RDW
Poikilocytosis

62
Q

Laboratory Signs of Hemolysis

A

Increased reticulocytes
Increased lactate dehydrogenase
Increased unconjugated bilirubin
Decreased haptoglobin

63
Q

Megaloblastic Cause

A

B12 and/or folic acid deficiency
Increase cell replication
Increase lactate dehydrogenase (LD)

64
Q

Megaloblastic Explanation

A

Nucleus lags behind cytoplasm in maturation
Cells grow larger without dividing

65
Q

Megaloblastic Characteristics/Blood Smear

A

Oval Macrocytes
Howell-Jolly Bodies
Hypersegmentation
Anisocytosis

66
Q

Iron deficiency Cause

A

Reduces erythropoietin production

67
Q

Iron deficiency Explanation

A

Cytoplasm lags behind nucleus in maturation due to inadequate iron for hgb synthesis

68
Q

Iron deficiency Characteristics

A

Microcytic, hypochromic RBCs

69
Q

Hemoglobin A

A

2 alpha, 2 beta chains
>95% adult reference range
20% newborn reference range

70
Q

Hemoglobin A2

A

2 alpha, 2 delta chains
1.5% - 3.7% adult reference range
<1% newborn reference range

71
Q

Hemoglobin F

A

2 alpha, 2 gamma chains
<2% adult reference range
50-85% newborn reference range

72
Q

Cellulose Acetate Agar

A
73
Q

Cellulose Acetate Hemoglobins

A

A
F
S and D
C and A2

74
Q

Cellulose Citrate Agar

A
75
Q

Cellulose Citrate Hemoglobins

A

S
C
A and D
F

76
Q

Sickle Cell Anemia (SS) Information

A

Inheritance of sickle cell gene from both parents
Bone marrow - hyperplasia
Retics 10-20%
Increase WBC
Increase platelet

77
Q

Sickle Cell Blood Smear

A

Anisocytosis
Poikilocytosis
Sickle Cells
Target Cells
nRBCs
Howell-Jolly bodies
Basophilic Stippling
Siderotic granules
Polychromasia

78
Q

Sickle Cell Hgb Electrophoresis

A

> 80% S
1-20% F
normal A2
no A

79
Q

Sickle Cell Trait (AS) Information

A

Inheritance of sickle cell gene from one parent
No anemia
Positive solubility

80
Q

Sickle Cell Trait (AS) Blood Smear

A

Occasional target cells
No sickle cells unless hypoxic

81
Q

Sickle Cell Trait (AS) Hgb Electrophoresis

A

50-65% A
35-45% S
normal F
N to slightly increase A2

82
Q

Hemoglobin C disease (CC) Information

A

Inheritance of gene for Hgb C from both parents
Mild to moderate anemia

83
Q

Hemoglobin C disease (CC) Blood Smear

A

Many target cells
Folded cells
Hgb C Crystals

84
Q

Hemoglobin C disease (CC) Hgb Electrophoresis

A

> 90% C
<7% F
no A

85
Q

Hemoglobin C Trait (AC) Information

A

Inheritance of gene for hgb C from 1 parent

86
Q

Hemoglobin C Trait (AC) Blood Smear

A

Many target cells

87
Q

Hemoglobin C Trait (AC) Hgb Electrophoresis

A

60-70% A
30-40% C

88
Q

Sickle Cell disease (SC) Information

A

Inheritance of 1 sickle cell & 1 hgb C gene
Positive Solubility Test
Mild to Moderate Anemia

89
Q

Sickle Cell disease (SC) Blood Smear

A

Many target cells
Folded & boat-shaped cells
SC crystals (finger-like projection)
Bizarre Shaped cells
Sickle Cells

90
Q

Sickle Cell disease (SC) Hgb Electrophoresis

A

50% S
50% C
Normal to 7% F
no A

91
Q

Hereditary Spherocytosis Information

A

Defect of cell membrane
MCHC usually >36 g/dl
Increase retics
Increase osmotic fragility

92
Q

Hereditary Spherocytosis Blood Smear

A

Spherocytes
Polychromasia

93
Q

Hereditary Spherocytosis Hgb Electrophoresis

A

Normal

94
Q

Autoimmune Hemolytic Anemia Information

A

Autoantibodies
Increase retics
Increase indirect bilirubin
Decrease haptoglobin
Positive DAT

95
Q

Autoimmune Hemolytic Anemia Blood Smear

A

Polychromasia
Spherocytes
nRBCs

96
Q

Autoimmune Hemolytic Anemia Hgb Electrophoresis

A

Normal

97
Q

Vitamin B12 Deficiency Information

A

Nutritional Deficiency
Malabsorption
Parasites
Pancytopenia

98
Q

Vitamin B12 Blood Smear

A

Oval macrocytes
Howell-Jolly Bodies
Hypersegmentation
Anisocytosis

99
Q

Pernicious anemia

A

Most common B12 deficiency
Lack of Intrinsic Factor

100
Q

Nonmegaloblastic Information

A

Alcoholism
Liver disease
Increase erythropoiesis
WBC & Platelet normal

101
Q

Nonmegaloblastic Blood Smear

A

Round macrocytes
No hypersegmentation

102
Q

Sideroblastic Anemia Information

A

Enzymatic defect in heme synthesis
RBC indices usually normal
Ringed Sideroblasts in marrow

103
Q

Sideroblastic Anemia Blood Smear

A

Dual population of RBCs (normocytic & microcytic)
Pappenheimer bodies
Basophilic Stippling

104
Q

Beta Thalassemia Major Information

A

Decrease Beta chain production
Severe anemia
MCV <67 fl
Homozygous

105
Q

Beta Thalassemia Major Blood Smear

A

Marked anisocytosis
Hypochromic microcytes
Target cells
Ovalocytes
nRBCs
Basophilic stippling

106
Q

Beta Thalassemia Major Hgb Electrophoresis

A

95-98% F
2-5% A2
Little to no A

107
Q

Beta Thalassemia Minor Information

A

Decrease Beta chain production
Heterozygous
Mild anemia

108
Q

Beta Thalassemia Minor Blood Smear

A

Anisocytosis
Hypochromic
Microcytes
Target Cells
Basophilic Stippling

109
Q

Beta Thalassemia Minor Hgb Electrophoresis

A

> 90-95% A
3.5-7% A2
2-5% F

110
Q

Iron deficiency CBC Information

A

Decrease RBCs
Increase RDW
Decrease Fe serum
Increase TIBC
Decrease Ferritin serum
Normal Hgb A2

111
Q

Sideroblastic Anemia CBC

A

Decrease RBCs
Increase RDW
Increase Fe serum
Normal TIBC
Increase Ferritin serum
Normal Hgb A2

112
Q

Beta Thalassemia Major CBC

A

Increase RBCs
Normal to Increase RDW
Increase Fe serum
Normal TIBC
Increase Ferritin serum
Decrease to absent Hgb A2

113
Q

Beta Thalassemia Minor CBC

A

Increase RBCs
Normal RDW
Normal Fe serum
Normal TIBC
Normal Ferritin serum
Increase Hgb A2

114
Q

Anemia of inflammation CBC

A

Decrease RBCs
Normal RDW
Decrease Fe serum
Decrease TIBC
Increase Ferritin serum
Normal Hgb A2

115
Q

Beta Thalassemia/Hemoglobin S Hgb Electrophoresis

A

50-95% Hgb S
0-50% Hgb A
2-30% Hgb F

116
Q

Hemolytic Uremic Syndrome (HUS) Blood Smear

A

Fragments
Burr Cells
Polychromasia