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Step 1 Immunology > Anemia > Flashcards

Anemia Flashcards

1
Q

MCV in microcytic anemia

A

Less than 80 μm3

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2
Q

MCV in macrocytic anemia

A

Greater than 100 μm3

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3
Q

Underlying etiology of microcytic anemia

A

underproduction of Hb causes “extra divisions” in precursors to maintain RBC Hb concentration

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4
Q

Underlying etiology of macrocytic anemia

A

Error in DNA synthesis causes “too few divisions” in precursors, which get stuck in G2

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5
Q

Underlying etiology of normocytic anemia

A

Destruction (peripheral and/or intravascular), or underproduction

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6
Q

Reticulocyte count in normocytic anemias

A

corrected retic >3%: destruction with normal marrow response

corrected retic

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7
Q

Cause of iron deficiency anemia in infants

A

Breast feeding

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8
Q

Cause of iron deficiency anemia in children

A

poor diet

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9
Q

Cause of iron deficiency anemia in adults

A

PUD in males
menorrhagia or pregnancy in females

Malabsorption (e.g. celiac’s)
Gastrectomy

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10
Q

Cause of iron deficiency anemia in elderly

A

Colon polyps/carcinoma, hookworm

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11
Q

Trace the path of iron from intake to storage (5 steps)

A

1) Intake via heme and non-heme forms
2) Duodenal enterocytes uptake via DMT1
3) Passed through enterocyte cytosol via ferroportin
4) Enters bloodstream attached to transferrin
5) Stored in hepatic and marrow macrophages via ferritin

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12
Q

Normal Hb in males

A

13.5 - 17.5

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13
Q

Normal Hb in females

A

12.5 - 16.0

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14
Q

Stages of iron deficiency, with [Ferritin, TIBC, Serum Fe, %saturation, RBC, and MCV] findings

A

1) Storage is depleted (↓Ferritin, ↑TIBC)
2) Serum is depleted (↓Serum Fe, ↓%saturation)
3) Normocytic anemia (↓RBC, MCV is NL)
4) Microcytic, hypochromic anemia (↓RBC, MCV)

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15
Q

Labs in IDA (RBC and serum)

A

1) Microcytic, hypochromic RBCs w/↑RDW

2) ↓Ferritin, ↑TIBC, ↓Serum Fe, ↓%sat, ↑FEP

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16
Q

Iron deficiency anemia with esophageal web and atrophic glossitis

A

Plummer-Vinson syndrome

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17
Q

MCV in normocytic anemia

A

80 - 100 μm3

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18
Q

Lab findings in anemia of chronic disease

A

↑Ferritin, ↓TIBC, ↓Serum Fe, ↓%saturation, ↑FEP

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19
Q

Tx of anemia in chronic kidney disease

A

EPO

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20
Q

Trace the path of iron from intake to storage (5 steps)

A

1) Intake via heme and non-heme forms
2) Duodenal enterocytes uptake via DMT1
3) Passed through enterocyte cytosol via ferroportin
4) Enters bloodstream attached to transferrin
5) Stored in hepatic and marrow macrophages via ferritin

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21
Q

Normal Hb types

A

HbA (α2 β2)
HbA2 (α2 δ2)
HbF (α2 γ2)

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22
Q

Why does gastrectomy cause IDA?

A

Higher pH results in higher proportion of Fe+3, which is less readily absorbed

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23
Q

Labs in IDA (RBC and serum)

A

1) Microcytic, hypochromic RBCs w/↑RDW

2) ↓Ferritin, ↑TIBC, ↓Serum Fe, ↓%sat, ↑FEP

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24
Q

Iron deficiency anemia Tx

A

Ferrous sulfate

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25
Q

Iron deficiency anemia Sxs (4)

A

Fatigue
Conjunctival pallor
Pica
Koilonychia

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26
Q

Heme synthesis pathway

A 
Succinyl CoA -[ALAS, B6]→
ALA -[ALAD]→
porphobilinogen -[in mitochondrion]→
protoporphyrin + Fe -[ferrocheletase]→
Heme
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27
Q

General etiology of sideroblastic anemias

A

Decreased protoporphyrin synthesis → decreased heme synthesis

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28
Q

General etiology of iron deficiency anemia

A

decreased iron intake → decreased heme synthesis

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29
Q

Why does sideroblastic anemia present with ringed sideroblasts in marrow?

A

iron collects in mitochondria, which circle erythroblast nuceus

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30
Q

What are the causes of sideroblastic anemia?

A 
Congenital ALAS deficiency
Alcoholism (mitochondrial poison)
Lead poisoning (inhibits ALAD and ferrochelatase)
Vitamin B6 deficiency (ALAS cofactor)
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31
Q

What anti-microbial can cause sideroblastic anemia?

A

Isoniazid

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32
Q

Lab findings in sideroblastic anemia

A

↑Ferritin, ↓TIBC, ↑Serum Fe, ↑%saturation (iron overload)

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33
Q

What is the mechanism by which chronic inflammation causes anemia?

A

Liver produces hepcidin, which inhibits:

1) Fe inhibits ferroportin @ macrophages and enterocytes
2) EPO production by kidney

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34
Q

Viral pathogen associated with severe Sxs in β-thalassemia major

A

Parvovirus B19 (aplastic crisis)

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35
Q

Tx of anemia in chronic kidney disease

A

EPO

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36
Q

General etiology of anemia in thalassemia?

A

Decreased globin production, due to congenital defects in genes encoding globin subunits

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37
Q

Thalassemia carriers are resistant to what infection?

A

Plasmodium falciparum

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38
Q

Thalassemia: Sxs associated with single α deletion

A

Asymptomatic

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39
Q

Thalassemia: Sxs associated with two α deletions

A

Mild anemia, ↑RBC

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40
Q

Agents used to treat lead poisoning

A

Dimercaprol and EDTA; succimer in kids

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41
Q

Hb type seen in α-thalassemia with three deletions

A

HbH (β4); damages RBC membranes

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42
Q

Thalassemia: Sxs associated with four α deletions

A

Hydrops fetalis, Hb Barts on electrophoresis

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43
Q

Hb type seen in α-thalassemia with four deletions

A

Hb Barts (γ4); damages RBC membranes

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44
Q

α deletion most prevalent in Asian populations

A

Cis deletion

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45
Q

General findings in megaloblastic anemia

A

macrocytic RBCs, hypersegmented PMNs, glossitis

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46
Q

Mutations causing β-thalassemia

A

Point mutations in splicing or promoter sites

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47
Q

β alleles

A

β0 - no production
β+ - reduced production
β - wild type

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48
Q

Sxs in β-thalassemia minor

A

Usually asymptomatic

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49
Q

Lab findings in β-thalassemia minor

A

Microcytic hypochromic anemia with target cells

Increased HbA2 and HbF

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50
Q

Sxs in β-thalassemia major

A

Severe anemia months after birth
Marrow expansion: “crew cut” skull x-ray, “chipmunk” facies
Extramedullary hematopoiesis: HSM
Chronic transfusions → 2ndary hemochromatosis

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51
Q

Lab findings in β-thalassemia major

A

Microcytic, hypochromic RBCs w/target cells and nucleated RBCs on smear
HbA2 and HbF, little to no HbA

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52
Q

Viral pathogen associated with severe Sxs in β-thalassemia major

A

Parvovirus B19 (aplastic crisis)

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53
Q

Mechanism of anemia in β-thalassemia major

A

Precipitation of unpaired α chains damages membranes, causing ineffective erythropoiesis and extravascular hemolysis

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54
Q

Phenotype for HbS/β-thalassemia heterozygote

A

mild to moderate sickle cell disease

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55
Q

“Burton lines”

A

Lead lines on gingiva

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56
Q

Demyelinating damage in B12 deficiency occurs at:

A

1) Spinocerebellar tract
2) Lateral corticospinal tract
3) Dorsal column

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57
Q

Type of anemia seen in lead poisoning

A

Microcytic sideroblastic anemia

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58
Q

Mechanism causing anemia in lead poisoning

A

Inhibition of ferrochelatase and ALAD causing decreased heme synthesis

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59
Q

Agents used to treat lead poisoning

A

Dimercaprol and EDTA; succimer in kids

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60
Q

Treatment for sideroblastic anemia

A

pyridoxine (B6)

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61
Q

Mechanism by which folate and B12 deficiency cause anemia

A

Folate is demethylated by B12, allowing it to participate in the production of DNA precursors. Deficiency in either inhibits DNA synthesis, which inhibits erythropoiesis

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62
Q

PMN findings and their cause in Megaloblastic anemia

A

Impaired maturation of granulocyte precursors due to deficient DNA synthesis causes hypersegmentation of PMN nuclei.

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63
Q

Findings in Diamond-Blackfan anemia

A

1) ↓Hb, ↑%HbF
2) Short stature
3) Craniofacial abnormalities
4) Triphalangeal thumbs

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64
Q

General findings in megaloblastic anemia

A

macrocytic RBCs, hypersegmented PMNs, glossitis

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65
Q

Folate intake and absorption

A

green vegetables and some fruits; absorbed in jejunum

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66
Q

Causes of folate deficiency (4)

A

Malnutrition (esp. alcoholism)
Malabsorption
Increased requirement (pregnancy, cancer, hemolytic anemia)
Drugs (esp. folate metabolism antagonists, phenytoin)

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67
Q

Clinical and lab findings in folate deficiency megaloblastic anemia (4)

A

1) macrocytic RBCs and hypersegmented PMNs
2) Glossitis
3) ↓ serum folate
4) ↑ homocysteine

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68
Q

Findings that differentiate folate deficiency from B12 deficiency

A

No neuro sxs, normal methylmalonic acid

69
Q

B12 intake and absorption pathway

A

1) intake via animal proteins
2) Salivary enzymes liberate B12
3) Salivary R-binder binds B12
4) Pancreatic proteases in duodenum liberate B12
5) Intrinsic factor (made by gastric parietal cells) binds B12 in the small bowel
6) IF-B12 complex absorbed in the ileum

70
Q

Cause of pernicious anemia

A

Anti-gastric parietal cell Abs cause IF deficiency, leading to B12 deficiency

71
Q

Causes of B12 deficiency (5)

A

1) Pernicious anemia
2) Poor intake (veganism, alcoholism)
3) Malabsorption in terminal ileum
4) Diphyllobothrium latum
5) Gastrectomy

72
Q

Clinical and lab findings in B12 deficiency

A

1) Macrocytic RBCs and hypersegmented PMNs
2) Glossitis
3) ↓Serum B12
4) ↑ homocysteine
5) ↑methylmalonic acid
6) Subacute degeneration of spinal cord→poor proprioception, poor vibration sensation, spastic paresis

73
Q

Cause of spinal degeneration in B12 deficiency

A

↑ Methylmalonic acid impairs myelinization

74
Q

Cause of ↑ risk of thrombosis in B12 and B9 deficiency

A

↑ homocysteine causes IV damage

75
Q

Demyelinating damage in B12 deficiency occurs at:

A

1) Spinocerebellar tract
2) Lateral corticospinal tract
3) Dorsal column

76
Q

Megaloblastic anemia with orotic acid in urine

A

Orotic aciduria

77
Q

Tx in hereditary spherocytosis?

A

Splenectomy

78
Q

Orotic aciduria presentation

A

Presents in childhood as:

1) Failure to thrive
2) Developmental delay
3) Megaloblastic anemia refractory to B12/B9 supplementation

79
Q

Lab finding differentiating between orotic aciduria and ornithine transcarbamylase deficiency

A

No hyperammonemia in orotic aciduria

80
Q

Tx in orotic aciduria

A

UMP

81
Q

Rapid onset of anemia in 1st year of life due to intrinsic defect in erythroid progenitor cells

A

Diamond-Blackfan anemia

82
Q

Findings in Diamond-Blackfan anemia

A

1) ↓Hb, ↑%HbF
2) Short stature
3) Craniofacial abnormalities
4) Triphalangeal thumbs

83
Q

Anemia and RBC macrocytosis without hypersegmented PMNs implies

A

No impairment of DNA synthesis

84
Q

Causes of nonmegaloblastic anemia

A

Liver disease, alcoholism, 5-FU

85
Q

Appearance of reticulocytes

A

large RBC with bluish tinge

86
Q

Reticulocyte count in normal marrow response to stress

A

> 3% (corrected)

87
Q

Site of RBC destruction in extravascular hemolysis

A

Reticuloendothelial macrophages in spleen, liver and lymph nodes

88
Q

Products of RBC destruction by macrophages

A

1) Globin → amino acids
2) Heme → Fe and protoporphyrin
3) Protoporphyrin → unconjugated bilirubin

89
Q

Excretion of unconjugated bilirubin is accomplished by:

A

1) Binding to serum albumin and delivery to liver
2) Conjugation to glucaronic acid to make water soluble conjugated bilirubin
3) Excretion of conjugated bilirubin into bile

90
Q

Clinical and lab findings in extravascular hemolysis (4)

A

1) Anemia w/spherocytes
2) ↑LDH
3) ↑ indirect bili → jaundice and bilirubin gallstones
4) Splenomegaly

91
Q

Clinical and lab findings in intravascular hemolysis (4)

A

1) Anemia w/schistocytes
2) ↑LDH
3) ↑ serum Hb, ↓ haptoglobin
4) Urine findings: hemoglobinuria, hemosiderinuria, urobilinogen in urine

92
Q

Phenotype in HbC/HbS heterozygotes

A

Milder sickle cell disease than HbS homozygotes

93
Q

Clinical and lab findings in hereditary spherocytosis (5)

A

1) Splenomegaly
2) Jaundice, ↑ indirect bili, and bilirubin gallstones
3) ↑LDH
4) Spherocytes, ↑MCHC
5) (+) osmotic fragility test

94
Q

Mechanism by which spherocytes are formed in hereditary spherocytosis

A

Defective cytoskeleton anchoring proteins → blebbing → loss of membrane

95
Q

Increase risk associated with pathogen in hereditary spherocytosis?

A

Aplastic crisis w/Parvovirus B19

96
Q

Tx in hereditary spherocytosis?

A

Splenectomy

97
Q

Presentation of PNH (3)

A

1) Hemoglobinuria in the morning (mild respiratory acidosis during sleep activates complement)
2) Pancytopenia
3) Venous thrombosis (esp. hepatic, portal, cerebral veins)

98
Q

Labs in PNH (6)

A

1) Hemoglobinemia
2) Hemoglobinuria
3) Hemosiderinuria (days later)
4) (+) sucrose test and (+) acidified serum test
5) (-) CD55 (DAF) on flow cytometry
6) Coombs (-)

99
Q

Sickle cell trait confers protection against

A

Plasmodium falciparum

100
Q

Cause of sickling?

A

Hypoxemia, dehydration or acidemia cause polymerization of HbS

101
Q

Sites of hemolysis in sickle cell anemia, and associated findings?

A

Extravascular: continuous sickling/unsickling damages RBC membranes, prompting removal at spleen
Associated findings: ↑ unconjugated bili → jaundice, gall stones

Intravascular: RBCs w/damaged membranes dehydrate
Associated findings: target cells, ↓haptoglobin

102
Q

What causes anemia in G6PD deficiency? (4)

A

Oxidative stress, e.g.

1) Sulfa drugs + dapsone
2) Antimalarials
3) infx
4) fava beans

103
Q

Signs of compensatory hyperhematopoiesis in sickle cell anemia

A

1) Chipmunk facies
2) “crew cut” skull x-ray
3) hepatomegaly

104
Q

Vaso-occlusive complications in sickle cell anemia (5)

A

1) Dactylitis (common presenting sign in infants)
2) Acute chest syndrome (pulmonary microocclusion)
3) Sequestration crises and Autosplenectomy w/Howell Jolly bodies → ↑infx by encapsulated organisms (esp. salmonella osteomyelitis)
4) Renal papillary necrosis w/hematuria
5) Pain crisis (e.g. priapism)

105
Q

Signs of acute chest syndrome in sickle cell anemia

A

Chest pain, SOB, lung infiltrates; most common cause of death in adults w/SSD
Often precipitated by PNA

106
Q

Increased risk associated with viral pathogen in SSD

A

Aplastic crisis w/Parvovirus B19

107
Q

Diagnosis in sickle cell trait

A

1) Metabisulfite screen

2) Hb electrophoresis

108
Q

Primary site of sx in sickle cell trait

A

Kidney: hypoxia and hypertonicity in medulla causes sickling → microinfarctions → microscopic hematuria

109
Q

Site of hemolysis in warm AIHA

A

Splenic macrophages eat IgG coated RBC membrane

110
Q

Clinical and lab findings in HbC disease

A

1) Mild anemia due to extravascular hemolysis

2) HbC crystals in RBCs

111
Q

Causes of warm AIHA (4)

A

1) SLE
2) CLL
3) α-methyldopa (self-antigen binding IgG)
4) Penicillin, cephalosporins (drug-membrane complex binding IgG)

112
Q

Complications of PNH (3)

A

1) Hepatic, portal and cerebral venous thrombosis
2) Progression to AML
3) Iron deficiency anemia

113
Q

Presentation of pyruvate kinase deficiency

A

hemolytic anemia in a newborn

114
Q

Proportion of Hb forms in sickle cell disease and trait

A

Disease: HbS - 90%; HbF - 8%; HbA2 - 2%
Train: HbA - 55%; HbS - 43%; HbA2 - 2%

115
Q

Defect in paroxysmal nocturnal hemoglobinuria

A

Acquired mutation in stem cell → loss of GPI anchor for DAF → disinhibition of complement mediated lysis of RBCs

116
Q

Presentation of PNH

A

1) Hemoglobinuria in the morning (mild respiratory acidosis during sleep activates complement)
2) Pancytopenia
3) Venous thrombosis (esp. hepatic, portal, cerebral veins)

117
Q

Labs in PNH

A

1) Hemoglobinemia

2)

118
Q

Tx in PNH

A

eculizumab (terminal complement inhibitor)

119
Q

Causes of cold AIHA (3)

A

1) CLL
2) Mycoplasma pneumoniae
3) Infectious mononucleosis

120
Q

G6PD role in oxidative stress

A

1) HMP shunt: G6P → G6PD
2) This regenerates NADPH
3) NADPH regenerates GSH
4) GSH inactivates free radicals/ROS

121
Q

What causes anemia in G6PD deficiency?

A

Oxidative stress, e.g.

1) Sulfa drugs + dapsone
2) Antimalarials
3) infx
4) fava beans

122
Q

G6PD deficiency presentation

A

Back pain and hemoglobinuria days after oxidative stress

123
Q

Populations with high prevalence of G6PD deficiency

A

1) African variant: mild reduction in G6PD half life → mild IV hemolysis
2) Mediterranean variant: marked reduction in G6PD half life → marked IV hemolysis

124
Q

What are Heinz bodies?

A

Oxidative stress → sulfur crosslinking in Hb → Hb precipitation; removal in spleen → bite cells

125
Q

When should enzyme studies by conducted in G6PD deficiency?

A

Weeks after the resolution of a hemolytic episode

126
Q

Cause of hemolysis in Autoimmune Hemolytic Anemia?

A

IgG or IgM antibody mediated destruction

127
Q

“Warm agglutinins”

A

anti-RBC IgG

128
Q

Site of hemolysis in warm AHA

A

Splenic macrophages eat IgG coated RBC membrane

129
Q

Blood smear in warm AIHA

A

Spherocytes

130
Q

Causes of warm AIHA

A

1) SLE
2) CLL
3) α-methyldopa (self-antigen binding IgG)
4) Penicillin, cephalosporins (drug-membrane complex binding IgG)

131
Q

Complications of PNH

A

1) Hepatic, portal and cerebral venous thrombosis
2) Progression to AML
3) Iron deficiency anemia

132
Q

Etiology of acute chest syndrome in SSD following PNA?

A

PNA → dilation of pulmonary vessels (↑transit time) + hypoxemia → sickling

133
Q

Tx in warm AIHA

A

DC causative drug, give steroids and IVIG, splenectomy if refractory

134
Q

Defect in Fanconi anemia

A

DNA repair defect → marrow failure

135
Q

Site of hemolysis in cold AIHA

A

Mild complement activation: RBCs inactivate complement, hemolysis @ spleen via C3b opsinization
Extreme complement activation: IV hemolysis

136
Q

Smear in cold AIHA

A

spherocytes

137
Q

Presentation in cold AIHA

A

Cold exposure → painful cyanotic fingers and toes

138
Q

Causes of cold AIHA

A

1) CLL
2) Mycoplasma pneumoniae
3) infectious mononucleosis

139
Q

Diagnostic testing for AIHA

A

Coombs testing

140
Q

Direct Coombs

A

Detects Ig/C3b coated RBCs

1) Coombs reagent (anti-Ig/C3b antibodies) added to patient blood
2) Agglutination occurs if RBCs are coated with Ig/C3b

141
Q

Indirect Coombs

A

Detects anti-RBC antibodies in serum

1) Coombs reagent and test RBCs added to patient serum
2) Agglutination occurs if serum contains anti-RBC antibodies

142
Q

Microangiopathic Hemolytic Anemia pathogenesis

A

RBCs are mechanically damaged in the circulation

143
Q

Causes of Microangiopathic Hemolytic Anemia (4)

A

1) TTP, HUS, HELLP
2) Prosthetic valves
3) Aortic stenosis
4) Malignant hypertension

144
Q

Blood smear in Microangiopathic Hemolytic Anemia

A

Schistocytes

145
Q

Complication of chronic Microangiopathic Hemolytic Anemia

A

Iron deficiency anemia

146
Q

Pathogenesis of anemia in TTP

A

anti-ADAMTS13 antibody → ↑vWF multimers → systemic microthrombi

147
Q

Pathogenesis of anemia in HELLP

A

idiopathic activation of coagulation cascade in pre-ecclampsia → system microthrombi

148
Q

Pathogenesis of anemia in HUS

A

Infx mediated damage to glomerular endothelium → glomerular microthrombi

149
Q

Infectious agents that directly rupture RBCs

A

Plasmodium, Babesia

150
Q

Pathogenesis of anemia in renal failure

A

↓EPO

151
Q

Pathogenesis of anemia in Parvovirus B19 infx

A

virus infects RBC progenitors, temporarily halting erythropoeisis

152
Q

Tx in aplastic anemia (4)

A

1) DC causative agents
2) Transfusions and GM-CSF/G-CSF
3) Immunosupression (idiopathic cases may be caused by abnormal T-cell activation)
4) Bone marrow transplant (last resort)

153
Q

Defect in Fanconi anemia

A

DNA repair

154
Q

Viral agents associated with aplastic anemia (4)

A

1) Parvovirus B19
2) EBV
3) HIV
4) Hepatitis

155
Q

Labs in aplastic anemia

A

↓reticulocytes, ↑EPO

156
Q

Sxs in aplastic anemia (4)

A

1) Fatigue, malaise
2) Pallor
3) Purpura, mucosal bleeding
4) Infection

157
Q

WBC in Cushing’s sydrome/corticosteroids

A

1) Eosinopenia (sequestered in lymph nodes)
2) Leukopenia (apoptosis)
3) Neutrophilia (disadhesion of marginated PMNs)

158
Q

Accumulated substrates in Pb poisoning

A

protoporphyrin, ALAD

159
Q

Accumulated substrates in Acute intermittent porphyria

A

porphobilinogen, ALAD, coporphobilinogen (urine)

160
Q

Accumulated substrate in porphyria cutanea tarda

A

Uroporphyrin

161
Q

Sxs of acute intermittent porphyria

A 
4 P's
Painful abdomen
Port-wine colored urine
Polyneuropathy
Psych disturbances
162
Q

Cause of acute intermittent porphyria

A

Defective porphobilinogen deaminase; precipitated by

1) Cytochrome P450 inducers
2) alcohol
3) Starvation

163
Q

Tx of acute intermittent porphyria

A

Glucose and heme (inhibit ALAS)

164
Q

Defect in porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase

165
Q

Sx of porphyria cutanea tarda

A

blistering cutaneous photosensitivity

166
Q

Mechanism of iron poisoning

A

Peroxidation of membrane lipids → cell death

167
Q

Sxs of iron poisoning

A

Nausea, vomiting, gastric bleeding, lethargy, GI scarring and obstruction

168
Q

Tx of iron poisoning

A

Dialysis and chelation therapy

1) IV deferoxamin
2) Oral deferasirox

Step 1 Immunology (13 decks)

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