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MICRO / PARA (DUMP) > AUTOIMMUNE > Flashcards

AUTOIMMUNE Flashcards

1
Q
  1. What is induced by immunization in the context of immune tolerance?
    a) Allergic response
    b) Immune activation
    c) Immune suppression
    d) Non-reactivity to self
A

d) Non-reactivity to self
Rationale: Immune tolerance refers to the immune system’s ability to not respond to certain antigens. Immunization can be used to induce this non-reactivity to self, thereby training the immune system to avoid responding to its own body’s cells and proteins.

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2
Q
  1. What is the primary location where self-reactive T cells are destroyed as a part of central tolerance?
    a) Bone marrow
    b) Liver
    c) Thymus
    d) Spleen
A

c) Thymus
Rationale: Central tolerance involves the elimination of self-reactive lymphocytes. For T cells, this process primarily takes place in the thymus.

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3
Q
  1. Where are self-reactive B cells typically destroyed?
    a) Thymus
    b) Spleen
    c) Bone marrow
    d) Lymph nodes
A

c) Bone marrow
Rationale: Central tolerance involves the elimination of self-reactive B cells primarily in the bone marrow.

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4
Q
  1. Which cells play a central role in maintaining the balance in peripheral tolerance?
    a) Memory B cells
    b) Neutrophils
    c) Regulatory T cells
    d) Basophils
A

c) Regulatory T cells
Rationale: Regulatory T cells, often referred to as Tregs, are essential for maintaining peripheral tolerance by ensuring a balance between different immune responses and preventing harmful autoimmune reactions.

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5
Q
  1. Th1 cells are primary mediators of which condition?
    a) Allergies
    b) Autoimmune diseases
    c) Bacterial infections
    d) Viral infections
A

b) Autoimmune diseases
Rationale: Th1 cells release proinflammatory cytokines and are major mediators of autoimmune diseases, where the immune system mistakenly attacks the body’s own cells.

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6
Q
  1. What do Th2 cells predominantly release?
    a) Proinflammatory cytokines
    b) Non-inflammatory cytokines
    c) Histamines
    d) Antibodies
A

b) Non-inflammatory cytokines
Rationale: Th2 cells are known to release cytokines that are not primarily inflammatory and are often involved in counteracting the proinflammatory response of Th1 cells.

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7
Q
  1. Loss of immune tolerance can lead to which of the following?
    a) Immunosuppression
    b) Autoimmune diseases
    c) Reduced antibody production
    d) Enhanced wound healing
A

b) Autoimmune diseases
Rationale: Loss of immune tolerance means the immune system might start reacting against self-antigens, which can lead to autoimmune diseases where the body attacks its own cells and tissues.

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8
Q
  1. Which describes the immune system’s random generation of sites directed against molecular configurations?
    a) Immune memory
    b) Antibody diversity
    c) Immune tolerance
    d) Peripheral defense
A

b) Antibody diversity
Rationale: The immune system’s ability to randomly generate various antibody sites ensures a wide range of antibody diversity, allowing it to recognize and combat a vast array of antigens.

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9
Q
  1. What can result when the immune system reacts with self-antigens?
    a) Immunodeficiency
    b) Enhanced immune memory
    c) Autoimmune diseases
    d) Immune acceleration
A

c) Autoimmune diseases
Rationale: If the immune system reacts with self-antigens, it can mistakenly attack the body’s own cells and tissues, leading to autoimmune diseases.

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10
Q
  1. Which cells release proinflammatory cytokines in response to foreign antigens?
    a) Th1
    b) Th2
    c) Memory B cells
    d) Basophils
A

a) Th1
Rationale: Th1 cells respond to foreign antigens by releasing proinflammatory cytokines to combat the perceived threat.

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11
Q
  1. Why do healthy individuals express detectable levels of autoimmune antibodies?
    a) To combat infections
    b) As a result of vaccine administration
    c) To maintain homeostasis
    d) Due to a genetic mutation
A

c) To maintain homeostasis
Rationale: Autoantibodies in healthy individuals can serve a regulatory function and play a role in maintaining homeostasis, ensuring the body’s internal environment remains stable.

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12
Q
  1. Which mechanism is responsible for removing aged red cells from human circulation?
    a) Immune tolerance
    b) Natural mechanism
    c) Peripheral defense
    d) Central tolerance
A

b) Natural mechanism
Rationale: Aged red cells are naturally removed from human circulation to maintain the health and functionality of the bloodstream.

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13
Q
  1. Which cells primarily mediate allergic reactions?
    a) Th1
    b) Th2
    c) Regulatory T cells
    d) Memory B cells
A

b) Th2
Rationale: Th2 cells are involved in immune responses against parasites and are also associated with allergic reactions.

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14
Q
  1. The immune system’s inability to attack its own cells and proteins is a description of:
    a) Immune activation
    b) Immune suppression
    c) Immune memory
    d) Immune tolerance
A

d) Immune tolerance
Rationale: Immune tolerance refers to the immune system’s ability to differentiate between foreign invaders and its own body, preventing it from attacking its own cells and proteins.

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15
Q
  1. Which organ is NOT directly involved in the process of central tolerance?
    a) Thymus
    b) Bone marrow
    c) Lymph nodes
    d) Liver
A

d) Liver
Rationale: Central tolerance involves the thymus (for T cells) and bone marrow (for B cells) in the elimination of self-reactive lymphocytes. The liver is not directly involved in this process.

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16
Q
  1. What causes damage in autoimmune diseases?
    a) Infections
    b) Foreign bodies
    c) Autoantibodies or autoreactive cells
    d) External injuries
A

c) Autoantibodies or autoreactive cells
Rationale: Autoimmune diseases result from the body’s immune system attacking its own tissues, which is mediated by autoantibodies or autoreactive cells.

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17
Q
  1. Which cell type is often involved in triggering autoimmune reactions?
    a) CD8 T-cells
    b) B-cells
    c) CD4 helper T-cells
    d) Macrophages
A

c) CD4 helper T-cells
Rationale: In autoimmune diseases, self-reactive CD4 helper T-cells are generally associated with inducing either cell-mediated or antibody-mediated autoimmune reactions.

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18
Q
  1. Which of the following is a systemic autoimmune disease?
    a) Multiple Sclerosis (MS)
    b) Myasthenia Gravis
    c) Systemic Lupus Erythematosus (SLE)
    d) Grave’s Disease
A

c) Systemic Lupus Erythematosus (SLE)
Rationale: Systemic Lupus Erythematosus (SLE) affects multiple organ systems, classifying it as a systemic autoimmune disease, whereas the others are more organ-specific.

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19
Q
  1. What is the primary treatment approach for Rheumatoid Arthritis (RA)?
    a) Hormone Replacement
    b) Anti-Inflammatory Drugs
    c) Cholinesterase Inhibitors
    d) Plasmapheresis
A

b) Anti-Inflammatory Drugs
Rationale: RA is primarily treated with anti-inflammatory drugs to reduce inflammation and pain associated with the condition.

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20
Q
  1. Which autoimmune disease involves the immune system attacking the thyroid cells, reducing thyroid hormone production?
    a) Grave’s Disease
    b) Multiple Sclerosis
    c) Pernicious Anemia
    d) Hashimoto’s Thyroiditis
A

d) Hashimoto’s Thyroiditis
Rationale: In Hashimoto’s Thyroiditis, the immune system targets the thyroid cells, leading to an inability of the thyroid gland to produce sufficient thyroid hormones.

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21
Q
  1. Which treatment is beneficial for Myasthenia Gravis?
    a) Splenectomy
    b) Cholinesterase Inhibitors and Thymectomy
    c) Plasmapheresis
    d) Metabolic Control
A

b) Cholinesterase Inhibitors and Thymectomy
Rationale: Myasthenia Gravis patients benefit from cholinesterase inhibitors (to improve muscle function) and thymectomy (removal of the thymus gland, which often shows abnormalities in these patients).

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22
Q
  1. Which autoimmune disease primarily affects the nervous system and leads to damage in the brain and spinal cord?
    a) Rheumatoid Arthritis (RA)
    b) Multiple Sclerosis (MS)
    c) Systemic Lupus Erythematosus (SLE)
    d) Grave’s Disease
A

b) Multiple Sclerosis (MS)
Rationale: Multiple Sclerosis (MS) targets the central nervous system, leading to inflammation, damage, and scarring of the myelin sheath in the brain and spinal cord.

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23
Q
  1. Which treatment approach involves the removal of certain components from blood?
    a) Anti-Inflammatory Drugs
    b) Splenectomy
    c) Plasmapheresis
    d) Hormone Replacement
A

c) Plasmapheresis
Rationale: Plasmapheresis is a process that filters the blood to remove harmful antibodies or other components, and it’s used in certain autoimmune conditions like SLE and GBS.

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24
Q
  1. Which autoimmune disease results in muscle weakness due to a breakdown in the communication between nerves and muscles?
    a) Myasthenia Gravis
    b) Pernicious Anemia
    c) Rheumatoid Arthritis (RA)
    d) Type 1 Diabetes
A

a) Myasthenia Gravis
Rationale: Myasthenia Gravis involves the production of antibodies that block or destroy muscle receptor sites, leading to a decline in muscle communication and resultant muscle weakness.

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25
Q
  1. Which condition involves the body’s immune system targeting the intrinsic factor, leading to a deficiency of vitamin B12?
    a) Hashimoto’s Thyroiditis
    b) Pernicious Anemia
    c) Grave’s Disease
    d) Good Pasture Syndrome
A

b) Pernicious Anemia
Rationale: Pernicious Anemia is caused when the body’s immune system targets the intrinsic factor (a protein essential for the absorption of vitamin B12), leading to a vitamin B12 deficiency.

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26
Q
  1. Which autoimmune disease primarily affects the synovial membrane leading to potential cartilage and bone damage?
    A. Systemic Lupus Erythematosus (SLE)
    B. Goodpasture Syndrome
    C. Rheumatoid Arthritis
    D. Myasthenia Gravis
A

C. Rheumatoid Arthritis
Rationale: Rheumatoid Arthritis is characterized by inflammation primarily in the synovial membrane which can lead to cartilage and bone damage.

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27
Q
  1. Which distinctive rash is a pathognomonic sign of SLE?
    A. Malar rash
    B. Discoid rash
    C. Roseola rash
    D. Ringworm rash
A

A. Malar rash
Rationale: A malar rash, or “butterfly rash”, is a pathognomonic sign of SLE. Its presence confirms the disease.

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28
Q
  1. Goodpasture Syndrome predominantly affects which two organs?
    A. Liver and Heart
    B. Lungs and Kidneys
    C. Skin and Brain
    D. Joints and Muscles
A

B. Lungs and Kidneys
Rationale: Goodpasture Syndrome affects the lungs and kidneys with the presence of antibodies targeting Type IV collagen.

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29
Q
  1. Which antibody is characteristically found in Rheumatoid Arthritis?
    A. Anti-dsDNA
    B. Rheumatoid factor (IgM against own IgG)
    C. Antibody directed at Type IV collagen
    D. Antinuclear antibody (ANA)
A

B. Rheumatoid factor (IgM against own IgG)
Rationale: Rheumatoid factor (IgM against one’s own IgG) is a characteristic feature of Rheumatoid Arthritis.

30
Q
  1. What do the initials ANA stand for in the context of autoimmune diseases?
    A. Anti-Nephrotic Antibody
    B. Antinuclear Antibody
    C. Anti-Neuralgic Acid
    D. Antinuclear Actin
A

B. Antinuclear Antibody
Rationale: ANA stands for Antinuclear Antibody, which is often associated with autoimmune diseases, especially SLE.

31
Q
  1. Which autoimmune disease involves the immune system attacking Type IV collagen?
    A. Rheumatoid Arthritis
    B. Systemic Lupus Erythematosus (SLE)
    C. Goodpasture Syndrome
    D. Myasthenia Gravis
A

C. Goodpasture Syndrome
Rationale: Goodpasture Syndrome is characterized by the presence of antibodies that target Type IV collagen, affecting the lungs and kidneys.

32
Q
  1. Which diagnostic criteria mnemonic is used for Systemic Lupus Erythematosus (SLE)?
    A. SOAP RASH MD
    B. SOAP BRAIN MD
    C. SLE CRITERIA
    D. LUPUS DIAGNOSE
A

B. SOAP BRAIN MD
Rationale: The mnemonic SOAP BRAIN MD is used to remember the diagnostic criteria for SLE.

33
Q
  1. In which autoimmune disease is there a hyperactive immune system with multiple abnormalities?
    A. Rheumatoid Arthritis
    B. Systemic Lupus Erythematosus (SLE)
    C. Goodpasture Syndrome
    D. Hashimoto’s Thyroiditis
A

B. Systemic Lupus Erythematosus (SLE)
Rationale: SLE demonstrates a hyperactive immune system with multiple abnormalities, characterized by a vast array of autoantibodies.

34
Q
  1. The butterfly rash, if present, confirms the diagnosis of which disease?
    A. Rheumatoid Arthritis
    B. Systemic Lupus Erythematosus (SLE)
    C. Goodpasture Syndrome
    D. Chronic (Hashimoto) Thyroiditis
A

B. Systemic Lupus Erythematosus (SLE)
Rationale: The butterfly rash, or malar rash, is a pathognomonic sign of SLE. Its presence confirms the disease.

35
Q
  1. How many symptoms from the SOAP BRAIN MD mnemonic are needed to formulate a diagnosis for SLE?
    A. 3 out of 11
    B. 4 out of 11
    C. 5 out of 11
    D. 6 out of 11
A

B. 4 out of 11
Rationale: To formulate a diagnosis for Systemic Lupus Erythematosus (SLE), 4 out of the 11 criteria from the SOAP BRAIN MD mnemonic are needed.

36
Q

Which autoimmune disease primarily targets myelin basic proteins in the CNS?
a) Myasthenia Gravis
b) Multiple Sclerosis
c) Hashimoto’s Thyroiditis
d) Guillain-Barre Syndrome

A

b) Multiple Sclerosis
Rationale: Multiple Sclerosis is characterized by the immune response against myelin basic proteins leading to demyelination in the CNS.

37
Q

In Myasthenia Gravis, the interaction with which neurotransmitter is blocked by antibodies?
a) Dopamine
b) Serotonin
c) GABA
d) Acetylcholine

A

d) Acetylcholine
Rationale: In Myasthenia Gravis, antibodies block acetylcholine receptors, causing faulty neuromuscular signal transmission.

38
Q

What is a primary manifestation of Hashimoto’s Thyroiditis?
a) Hyperthyroidism
b) Hypothyroidism
c) Hypoglycemia
d) Hyperglycemia

A

b) Hypothyroidism
Rationale: Hashimoto’s Thyroiditis is characterized by an autoimmune attack on the thyroid gland, leading to reduced production of thyroid hormones and causing hypothyroidism.

39
Q

Which disease involves antibodies that block thyroid-stimulating hormone receptors?
a) Grave’s Disease
b) Type 1 Diabetes
c) Pernicious Anemia
d) Guillain-Barre Syndrome

A

a) Grave’s Disease
Rationale: Grave’s Disease is characterized by antibodies that block thyroid-stimulating hormone receptors, leading to hyperthyroidism.

40
Q

Type 1 Diabetes is primarily due to the destruction of which cells in the pancreas?
a) Alpha cells
b) Delta cells
c) Beta cells
d) Gamma cells

A

c) Beta cells
Rationale: Type 1 Diabetes is a result of the immune system destroying the Beta cells of the pancreas, which produce insulin.

41
Q

In Pernicious Anemia, the malabsorption of which vitamin is a primary issue?
a) Vitamin A
b) Vitamin C
c) Vitamin D
d) Vitamin B12

A

d) Vitamin B12
Rationale: Pernicious Anemia is characterized by the inability to absorb vitamin B12 due to the loss of intrinsic factor.

42
Q

Which disorder is associated with the immune-mediated destruction of platelets?
a) Myasthenia Gravis
b) Grave’s Disease
c) Idiopathic Thrombocytopenia Purpura
d) Multiple Sclerosis

A

c) Idiopathic Thrombocytopenia Purpura
Rationale: ITP is characterized by antibody-mediated platelet destruction, leading to symptoms like petechiae and bleeding issues.

43
Q

Guillain-Barre Syndrome is predominantly mediated by which type of cells?
a) B cells
b) T cells
c) Plasma cells
d) Mast cells

A

b) T cells
Rationale: Guillain-Barre Syndrome involves an immune reaction against myelin, and it is mainly T-cell mediated.

44
Q

Which autoimmune disorder has muscle weakness and fatigue as its primary manifestations?
a) Myasthenia Gravis
b) Grave’s Disease
c) Multiple Sclerosis
d) Pernicious Anemia

A

a) Myasthenia Gravis
Rationale: Myasthenia Gravis is characterized by faulty neuromuscular signal transmission due to antibodies blocking acetylcholine receptors, leading to muscle weakness and fatigue.

45
Q

Which of the following disorders results from an immune response to Type IV collagen?
a) Multiple Sclerosis
b) Guillain-Barre Syndrome
c) Goodpasture Syndrome
d) Grave’s Disease

A

c) Goodpasture Syndrome
Rationale: Goodpasture Syndrome involves an immune attack on Type IV collagen, affecting both the lungs and kidneys.

46
Q

Which disease is characterized by a “butterfly rash” on the face?
a) Guillain-Barre Syndrome
b) Multiple Sclerosis
c) Systemic Lupus Erythematosus (SLE)
d) Grave’s Disease

A

c) Systemic Lupus Erythematosus (SLE)
Rationale: The butterfly rash is a pathognomonic sign of SLE, indicating its presence with certainty when observed.

47
Q

In which condition are antibodies produced against thyroglobulin (anti-Tg) and thyroid peroxidase (anti-TPO) antigens?
a) Myasthenia Gravis
b) Pernicious Anemia
c) Chronic Thyroiditis (Hashimoto)
d) Type 1 Diabetes

A

c) Chronic Thyroiditis (Hashimoto)
Rationale: Hashimoto’s Thyroiditis involves the production of autoantibodies directed against thyroglobulin and thyroid peroxidase antigens, leading to hypothyroidism.

48
Q

Which autoimmune disorder is associated with leg weakness that progresses proximally?
a) Guillain-Barre Syndrome
b) Multiple Sclerosis
c) Grave’s Disease
d) Myasthenia Gravis

A

a) Guillain-Barre Syndrome
Rationale: Guillain-Barre Syndrome manifests as leg weakness that gradually progresses upwards to affect other body parts, including the arms and face.

49
Q

Which autoimmune disorder leads to hyperthyroidism due to antibodies mimicking thyrotropin?
a) Multiple Sclerosis
b) Grave’s Disease
c) Guillain-Barre Syndrome
d) Chronic Thyroiditis (Hashimoto)

A

b) Grave’s Disease
Rationale: Grave’s Disease involves antibodies that mimic thyrotropin, leading to the proliferation of thyroid cells and hyperthyroidism.

50
Q

Which autoimmune disease results in the inability to synthesize insulin?
a) Guillain-Barre Syndrome
b) Grave’s Disease
c) Type 1 Diabetes
d) Pernicious Anemia

A

c) Type 1 Diabetes
Rationale: Type 1 Diabetes arises from the immune-mediated destruction of the Beta cells in the pancreas, leading to the inability to produce insulin.

51
Q

Which disorder is characterized by the absence of normal thymic tissue and severe infections due to a failure in the immune system?
a. DiGeorge Syndrome
b. X-linked Agammaglobulinemia
c. Chronic Granulomatous Disease
d. Severe Combined Immunodeficiency (SCID)

A

d. Severe Combined Immunodeficiency (SCID)

Rationale: SCID is defined by the absence of normal thymic tissue and the failure of both the cellular and humoral components of the immune system, leading to severe infections.

52
Q

Which disorder is linked with chromosome 22q11 deletions and cardiac defects?
a. Common Variable Immunodeficiency
b. DiGeorge Syndrome
c. ADA Deficiency
d. Hyper-IgE Immunodeficiency

A

b. DiGeorge Syndrome

Rationale: DiGeorge Syndrome results from defective embryonic development affecting the thymus, parathyroids, and cardiac outflow tract. It is associated with chromosome 22q11 deletions.

53
Q

Which immunodeficiency primarily presents in early childhood with recurrent infections mainly from encapsulated bacteria?
a. ADA Deficiency
b. Hyper-IgE Immunodeficiency
c. X-linked Agammaglobulinemia
d. Hyper-IgM Immunodeficiency

A

c. X-linked Agammaglobulinemia

Rationale: X-linked Agammaglobulinemia presents in early childhood with recurrent infections, especially from encapsulated bacteria, due to a defect in the pre-B lymphocyte maturation stage.

54
Q

ADA deficiency leads to the accumulation of which toxic metabolites?
a. Intracellular purine
b. Adenosine
c. IgE
d. IgA

A

b. Adenosine

Rationale: ADA Deficiency results in the accumulation of toxic adenosine metabolites due to a malfunction of the ADA enzyme in the purine salvage pathway.

55
Q

Which disease is an X-linked disorder that affects granulocyte function and presents with recurrent skin infections?
a. Selective IgA Deficiency
b. Hyper-IgM Immunodeficiency
c. Chronic Granulomatous Disease
d. Hyper-IgE Immunodeficiency

A

c. Chronic Granulomatous Disease

Rationale: Chronic Granulomatous Disease is X-linked, affecting granulocyte function, leading to recurrent skin infections, abscesses, and granulomas.

56
Q

Which immunodeficiency disorder results in a marked reduction in antibody production and is associated with recurrent sinopulmonary infections?
a. ADA Deficiency
b. Common Variable Immunodeficiency
c. DiGeorge Syndrome
d. X-linked Agammaglobulinemia

A

b. Common Variable Immunodeficiency

Rationale: Common Variable Immunodeficiency’s primary immunologic abnormality is a significant reduction in antibody production, leading to recurrent infections like sinusitis, otitis, and pneumonia.

57
Q

Which disorder is characterized by notably high IgE levels and symptoms like eczematous dermatitis and osteopenia?
a. Chronic Granulomatous Disease
b. Hyper-IgE Immunodeficiency
c. DiGeorge Syndrome
d. Hyper-IgM Immunodeficiency

A

b. Hyper-IgE Immunodeficiency

Rationale: Hyper-IgE Immunodeficiency is defined by extremely high IgE levels and various symptoms, including eczematous dermatitis, osteopenia, and more.

58
Q

In which disorder are Serum IgA levels markedly depressed, often less than 5 mg/dL?
a. X-linked Agammaglobulinemia
b. ADA Deficiency
c. Selective IgA Deficiency
d. Hyper-IgM Immunodeficiency

A

c. Selective IgA Deficiency

Rationale: Selective IgA Deficiency is characterized by severely reduced serum IgA levels, often dropping below 5 mg/dL.

59
Q

Which immunodeficiency results from a retroviral infection and leads to severe CD4 T lymphocyte dysfunction?
a. Common Variable Immunodeficiency
b. Hyper-IgE Immunodeficiency
c. ADA Deficiency
d. Acquired Immunodeficiency Syndrome (AIDS)

A

d. Acquired Immunodeficiency Syndrome (AIDS)

Rationale: AIDS is caused by the HIV retrovirus, resulting in severe CD4 T lymphocyte dysfunction, opportunistic infections, and malignancies.

60
Q

Which enzyme is responsible for the metabolism of adenosine in the purine salvage pathway?
a. Catalase
b. NADPH oxidase
c. Adenosine Deaminase (ADA)
d. Purine Nucleoside Phosphorylase (PNP)

A

c. Adenosine Deaminase (ADA)

Rationale: ADA is an enzyme in the purine salvage pathway responsible for metabolizing adenosine.

61
Q

Which disorder is characterized by a defect that results in the accumulation of intracellular purine metabolites, affecting T lymphocyte function?
a. Hyper-IgM Immunodeficiency
b. Chronic Granulomatous Disease
c. Purine Nucleoside Phosphorylase (PNP) Deficiency
d. ADA Deficiency

A

c. Purine Nucleoside Phosphorylase (PNP) Deficiency

Rationale: PNP Deficiency leads to the accumulation of intracellular purine metabolites due to depressed PNP activity, resulting in functional abnormalities in T lymphocytes.

62
Q

Which disease is known for its defective gene coding that inhibits oxidative metabolism, compromising neutrophil killing activity?
a. Selective IgA Deficiency
b. Chronic Granulomatous Disease
c. Hyper-IgE Immunodeficiency
d. Common Variable Immunodeficiency

A

b. Chronic Granulomatous Disease

Rationale: Chronic Granulomatous Disease has defects in the gene coding for NADPH oxidase, which compromises the neutrophil killing activity due to inhibited oxidative metabolism.

63
Q

Which disorder leads to increased production of IgM but no production of IgG or IgA due to defective expression of CD154?
a. Hyper-IgE Immunodeficiency
b. Hyper-IgM Immunodeficiency
c. ADA Deficiency
d. Common Variable Immunodeficiency

A

b. Hyper-IgM Immunodeficiency

Rationale: Hyper-IgM Immunodeficiency is characterized by the defective expression of CD154, which impairs B-cell isotype switching, resulting in the production of IgM but not IgG or IgA.

64
Q

What is the most common opportunistic infection associated with HIV infection?
a. Kaposi’s sarcoma
b. Oral candidiasis (thrush)
c. Pneumocystis jirovecii lung infection
d. Hairy leukoplakia

A

c. Pneumocystis jirovecii lung infection

Rationale: The lung infection with Pneumocystis jirovecii is the most common opportunistic infection associated with HIV infection.

65
Q

Which disease is characterized by profound T lymphocytopenia due to thymic aplasia and is not associated with a significant decrease in B lymphocytes or immunoglobulin production in most patients?
a. Common Variable Immunodeficiency
b. ADA Deficiency
c. X-linked Agammaglobulinemia
d. Congenital Thymic Aplasia (DiGeorge Syndrome)

A

d. Congenital Thymic Aplasia (DiGeorge Syndrome)

Rationale: DiGeorge Syndrome results in profound T lymphocytopenia due to thymic aplasia, but in most patients, B lymphocytes and immunoglobulin production are unaffected.

66
Q

Which enzyme deficiency leads to extreme cytopenia of both B and T lymphocytes due to the buildup of toxic metabolites?
a. Catalase
b. Adenosine Deaminase (ADA)
c. Purine Nucleoside Phosphorylase (PNP)
d. NADPH oxidase

A

b. Adenosine Deaminase (ADA)

Rationale: ADA deficiency results in the accumulation of toxic adenosine metabolites, leading to extreme cytopenia of both B and T lymphocytes.

67
Q

Which disorder commonly presents with GI malabsorption, autoimmune disorders, and neoplasms and is the most common primary immune deficiency disorder in adults?
a. Common Variable Immunodeficiency
b. Hyper-IgE Immunodeficiency
c. ADA Deficiency
d. X-linked Agammaglobulinemia

A

a. Common Variable Immunodeficiency

Rationale: Common Variable Immunodeficiency is the most common primary immune deficiency in adults and is associated with various complications including GI malabsorption, autoimmune disorders, and neoplasms.

68
Q

Which disorder results in patients being very susceptible to viruses but generally not to parasites?
a. Immunodeficiency disorders
b. Hyper-IgE Immunodeficiency
c. ADA Deficiency
d. Chronic Granulomatous Disease

A

a. Immunodeficiency disorders

Rationale: Immunodeficiency disorders, in general, make patients susceptible to viruses but usually not to parasites. However, exceptions include disorders like X-linked agammaglobulinemia and common variable immunodeficiency.

69
Q

Which of the following organisms is the most common pathogen associated with Chronic Granulomatous Disease?
a. H. influenza
b. S. pneumoniae
c. S. aureus
d. C. albicans

A

c. S. aureus

Rationale: In patients with Chronic Granulomatous Disease, S. aureus is the most commonly identified pathogen.

70
Q

In which immunodeficiency disorder are IgA levels extremely low, leading to an increased risk for transfusion reactions to certain blood products?
a. X-linked Agammaglobulinemia
b. Hyper-IgM Immunodeficiency
c. ADA Deficiency
d. Selective IgA Deficiency

A

d. Selective IgA Deficiency

Rationale: Selective IgA Deficiency

MICRO / PARA (DUMP) (12 decks)

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