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Systems Pathology II Final > Red Blood Cell Disorders 2 > Flashcards

Red Blood Cell Disorders 2 Flashcards

1
Q

What is the function of glutathione in the vascular system?

A

Neutralizes oxidants that may cause hemolysis

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2
Q

What is the result of reduced glutathione (GSH)?

A

RBC oxidative stress resulting in damage and hemolysis

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3
Q

What condition is a deficiency in the enzyme needed for the synthesis of glutathione?

A

Glucose-6-phosphate dehydrogenase deficiency (G6PD)

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4
Q

What is the enzyme responsible for GSH synthesis?

A

G6PD

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5
Q

What gender is most commonly affected by G6PD deficiency and why?

A

Males (X-linked condition)

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6
Q

What is the most common method of decreased glutathione production?

A

G6PD deficiency

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7
Q

Although asymptomatic usually, when does G6PD deficiency become symptomatic?

A

During exposure to an environmental stimulus that creates oxidative stress

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8
Q

What is the most common thing that causes symptoms of G6PD deficiency?

A

Infections

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9
Q

What general things can cause symptoms of G6PD deficiency?

A

1 infections
2 ADRs
3 Fava beans

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10
Q

What types of medications can cause ADRs and lead to symptoms of G6PD deficiency?

A

Aspirin, antimalarial meds (primaquine), nitrofurantoin (antibiotic for E. coli UTIs)

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11
Q

Heinz bodies and bite cells are microscopic findings associated with what hemopoietic condition?

A

Glucose-6-phosphate dehydrogenase deficiency

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12
Q

What race is more at risk for developing G6PD deficiency?

A

Africans (10% of African Americans)

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13
Q

What are the risks for G6PD deficiency?

A

Males, Africans, areas of endemic malaria

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14
Q

What condition involves complement-mediated hemolysis?

A

Paroxysmal nocturnal hemoglobinuria (PNH) or immunohemolytic anemias

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15
Q

What type of mutation is seen with PNH?

A

Acquired mutation of the PIGA gene in myeloid stem cells that is X-linked

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16
Q

What is the treatment for PNH?

A

Antibodies that inhibit the MAC

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17
Q

What is the characteristic sign of PNH?

A

Dark urine upon waking

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18
Q

In immunohemolytic anemias, what performs the hemolysis?

A

Splenic macrophages

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19
Q

How are immunohemolytic anemias diagnosed?

A

Coombs antiglobulin test

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20
Q

What class of antibodies are involved with attacking RBCs in warm antibody immunohemolytic anemia? Cold antibody?

A 
Warm = IgG
Cold = IgM
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21
Q

Which type of immunohemolytic anemia is usually primary in nature? Which can be secondary to infection?

A 
Warm = primary
Cold = primary or secondary to infection like EBV, mycoplasma, or B cell lymphomas
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22
Q

In 25% of cases, warm antibody immunohemolytic anemia can develop secondary to what conditions?

A

SLE, ADRs, B cell leukemia

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23
Q

What are the symptoms and signs of immunohemolytic anemias?

A

Chronic/mild anemia, jaundice, Raynaud phenomenon

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24
Q

What is the treatment for immunohemolytic anemias?

A

Usually none required

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25
Q

What kinds of things can cause traumatic hemolysis?

A

Prosthetic heart valves (“blender effect”) or narrowing vasculature

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26
Q

What kinds of things can cause narrowing of the vasculature resulting in traumatic hemolysis?

A

Aortic valve stenosis, DIC, SLE, invasive cancer

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27
Q

Microangiopathic hemolytic anemia is another name for what kind of hemolysis?

A

Traumatic hemolysis

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28
Q

What are RBCs called after damage due to traumatic hemolysis?

A

Schistocytes

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29
Q

What are other names for schistocytes based on their appearance?

A

“Burr cells” or “helmet cells”

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30
Q

Malaria is most common on which continents?

A

Africa and Asia

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31
Q

How many people die per year from malaria?

A

1 million

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32
Q

What is the pathogen responsible for malaria?

A

Plasmodium falciparum (protozoan)

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33
Q

What is the vector for malaria? What is the sole reservoir?

A 
Vector = female Anopheles mosquito
Reservoir = humans
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34
Q

What is the name for the hemolytic anemia associated with malaria?

A

Blackwater fever

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35
Q

Malaria becomes most lethal when it invades which body system?

A

CNS (seizures, convulsions, coma/death)

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36
Q

What is the treatment for malaria?

A

Chloroquine, primaquine, chemotherapy

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37
Q

Why is malaria becoming difficult to treat?

A

Drug-resistance

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38
Q

Deficiencies in which vitamins can lead to megaloblastic anemia?

A

Folic acid or vitamin B12

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39
Q

What is aplastic anemia?

A

Bone marrow failure

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40
Q

What is myelopththisic anemia?

A

Marrow infiltration

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41
Q

What is the effect of anemia of chronic disease?

A

Systemic inflammation

42
Q

What four types of anemias are anemias of diminished erythropoiesis?

A

1 nutritional deficiencies
2 anemia of chronic disease
3 aplastic anemia
4 myelopththisic anemia

43
Q

What is the prevalence of iron deficiency anemia in both developed and undeveloped countries?

A 
DevelopING = 25-50%
DevelopED = 10%
44
Q

What is the overall most common cause of anemia?

A

Iron deficiency usually through nutritional deficiency

45
Q

Where is 80% of iron stored?

A

Hemoglobin

46
Q

Where do we find our iron stores?

A

Liver, spleen, marrow, skeletal muscle

47
Q

What protein transports iron?

A

Transferrin (serum ferritin)

48
Q

What is the appearance of RBCs with iron deficiency anemia?

A

Microcytic and hypochromic

49
Q

What is usually the reason for iron deficiency in developed nations? In developing nations?

A 
DevelopED = chronic blood loss (abnormalities, GI issues, gynecological issues)
DevelopING = decreased dietary intake
50
Q

What are the 4 general causes of iron deficiency?

A

1 decreased dietary intake
2 chronic blood loss
3 malabsorption
4 increased metabolic demands (pregnancy or infancy)

51
Q

What kinds of malabsorptive conditions can lead to iron deficiency?

A

Caron’s disease, celiac disease, ulcerative colitis, etc.

52
Q

What is the treatment for iron deficiency anemia?

A

Iron supplementation

53
Q

How is iron deficiency anemia diagnosed?

A

Low serum ferritin levels and high EPO levels

54
Q

What are the signs and symptoms of iron deficiency anemia?

A

Fatigue, pale skin, impaired cognition, decreased immunity, fingernail spooning, pica

55
Q

What is pica?

A

Appetite for substances of non-nutritive value (example = dirt)

56
Q

Spooning of the fingernails would be a sign of what condition?

A

Iron deficiency anemia

57
Q

Chronic blood loss can be caused by what conditions of the GI tract?

A

Ulcers, colon cancer, hemorrhoids, celiac, ulcerative colitis

58
Q

Chronic blood loss can be caused by what conditions of the female genital tract?

A

Menorrhagia, metrorrhagia, leiomyoma, leiomyosarcoma

59
Q

Folate and vitamin B12 are required for what important function in the body?

A

DNA synthesis

60
Q

What is the result of megaloblastic anemia?

A

Inadequate DNA replication, production of megaloblasts, pancytopenia

61
Q

What is pancytopenia?

A

Decreased RBCs, WBCs, and platelets (anemia, leukopenia, and thrombocytopenia)

62
Q

Which type of nutritive anemia is NOT associated with neuralgic dysfunction: folate or vitamin B12?

A

Folate

63
Q

What happens to folic acid after cooking?

A

Denatures 10-15 minutes after cooking

64
Q

How is folate deficiency anemia diagnosed?

A

Macrocytes in peripheral blood, decreased serum folate, normal vitamin B12

65
Q

What is another name for vitamin B12 deficiency anemia?

A

Pernicious anemia

66
Q

What are the functions of vitamin B12?

A

DNA synthesis

Maintenance of PNS and spinal cord

67
Q

What population is basically the only group that would be at risk of vitamin B12 deficiency anemia via diet?

A

Strict vegans

68
Q

What is the most common cause of pernicious anemia?

A

Chronic malabsorption

69
Q

How does chronic malabsorption lead to pernicious anemia?

A

Gastric mucosal atrophy due to decreased intrinsic factor

70
Q

Pernicious anemia stems from an autoimmune disorder that attacks which specific things?

A

Parietal cells and intrinsic factor

71
Q

Pernicious anemia is most common among what population?

A

Elderly (over 70 years)

72
Q

What neurologic effect can be seen with vitamin B12 deficiency anemia?

A

Possible demyelination of the PNS and spinal cord (CNS)

73
Q

What symptoms can be indicative of neuropathy associated with pernicious anemia?

A

Numbness, tingling, burning, ataxia

74
Q

How is vitamin B12 deficiency anemia diagnosed?

A

Decreased serum vitamin B12, normal folate levels

75
Q

What is the treatment for pernicious anemia?

A

Vitamin B12 therapy (leads to rapid recovery)

76
Q

Even with treatment, which symptoms of pernicious anemia are unlikely to be resolved?

A

Neurological symptoms

77
Q

Vitamin B12 deficiency anemia is associated with an increased risk of what type of cancer?

A

Gastric carcinoma

78
Q

Anemia of chronic disease is most common among what population?

A

Hospitalized

79
Q

What effect does inflammation have on erythropoiesis?

A

Decreases it

80
Q

What 3 general categories of illness can cause chronic/systemic inflammatory disorders leading to decreased erythropoiesis?

A

1 microbial infection (osteomyelitis, lung abscess)
2 immune disorders ( RA, Crohn disease)
3 neoplasia (lymphoma, lung or breast cancer)

81
Q

What are the features of anemia of chronic disease?

A

1 decreased iron-binding capacity
2 increased iron storage in marrow
3 serum ferritin

82
Q

What is important about the features of anemia of chronic disease?

A

Used to rule out iron deficiency anemia

83
Q

What is aplastic anemia?

A

Suppression of myeloid stem cells (bone marrow failure)

84
Q

What kinds of viral infections present as an increased risk for aplastic anemia?

A

EBV, CMV, VZV

85
Q

In what way is aplastic anemia autoimmune?

A

Autoreactive T cells attack marrow

86
Q

What is the prognosis for aplastic anemia?

A

Unpredictable (worse when the cause is idiopathic)

87
Q

What is a visible sign of thrombocytopenia commonly seen with aplastic anemia?

A

Petechiae

88
Q

What class anemia symptom is characteristically absent in aplastic anemia?

A

Splenomegaly

89
Q

What is the treatment for aplastic anemia?

A

Immunosuppressive meds (80% response rate)

90
Q

What is the appearance of RBCs with aplastic anemia?

A

Normocytic, normochromic (reticulocytopenia, as well)

91
Q

Myelophthisic anemia most commonly metastasizes to what location?

A

Bone (also to breast, lung, and prostate)

92
Q

Myelophthisic anemia is seen in which conditions?

A

Chronic granulomatous disease (TB, Bridges-Good syndrome)

Lipid-storage disease (Niemann-Pick, type C)

93
Q

Dacrocytes are a finding with what type of anemia?

A

Myelophthisic anemia

94
Q

What is the treatment for myelophthisic anemia?

A

Marrow transplant

95
Q

What is the difference between relative and absolute polycythemia?

A

Relative - result of decreased plasma

Absolute - increase of actual total RBC mass

96
Q

Dehydration from vomiting, diarrhea, burns, and diuretics would cause what type of polycythemia?

A

Relative (due to decreased overall plasma)

97
Q

What things could cause absolute polycythemia?

A

1 proliferation of myeloid stem cells (polycythemia vera)

2 increased EPO (hypoxia, altitude, exogenous EPO)

98
Q

What is polycythemia vera?

A

Blood cancer with NO increase in EPO

99
Q

Which leads to increased EPO: hypoxia, anemia, or polycythemia vera?

A

Both hypoxia and anemia

100
Q

Which leads to decreased EPO: hypoxia, anemia, or polycythemia vera?

A

PCV

101
Q

What are the features of polycythemia?

A

Pruritis of the skin, DVT, PE, TIA/stroke

Systems Pathology II Final (8 decks)

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