1
Q
Which immunodeficiency is caused by CD154 deficiency
A
X-linked hyper IgM syndrome
2
Q
Which condition is due to neutrophil production abnormality
A
Congenital neutropenia
3
Q
Which disease is caused by NADPH oxidase deficiency
A
Chronic Granulomatous Disease
4
Q
Adenosine deaminase deficiency leads to which condition
A
Severe Combined Immunodeficiency SCID
5
Q
Failure of T cells to become polarized is seen in which syndrome
A
Wiskott Aldrich syndrome
6
Q
Absence of counter receptors to E and P selectins causes which disease
A
Leukocyte Adhesion Deficiency LAD
7
Q
Why do individuals with SCID fail to make antibody responses to vaccines
A
Most antibody responses require T cell help for B cells
8
Q
Why do infants with XLA only show symptoms after 4 to 6 months
A
Maternal IgG provides early protection
9
Q
Why are protein vaccine responses impaired in Wiskott Aldrich syndrome
A
Defective CD4 T cell polarization
10
Q
Why is a very rapid infection within 2 days unlikely to be B or T cell defect
A
Adaptive immunity is too slow to respond that quickly
11
Q
Which condition does NOT impair macrophage activation
A
Interleukin 10 deficiency
12
Q
What CD4 count defines AIDS
A
Below 200 cells per microliter
13
Q
What does SPUR stand for
A
Severe Persistent Unusual and Recurrent infections
14
Q
What is the risk window after a corticosteroid burst
A
90 to 180 days increased infection risk
15
Q
A child with granulomas and abscesses likely has which enzyme defect
A
NADPH oxidase deficiency
16
Q
At what age can selective IgA deficiency be diagnosed
A
Older than 4 years
17
Q
Which complement deficiency causes recurrent Neisseria infections
A
C5 to C9 deficiency
18
Q
Secondary immunodeficiencies are more common than primary
A
t
19
Q
CVID increases risk of lymphoma and gastric cancer
A
t
20
Q
Antigen specific antibody deficiency affects response to which microbes
A
Polysaccharide encapsulated microbes
21
Q
What is the difference between primary and secondary immunodeficiency
A
Primary is genetic secondary is acquired
22
Q
Which gender is affected by X linked agammaglobulinemia
A
Males
23
Q
Why does XLA present after 6 months
A
Maternal IgG declines
24
Q
What enzyme is defective in CGD
A
NADPH oxidase
25
Delayed umbilical cord separation is seen in which disease
Leukocyte Adhesion Deficiency
26
What is the triad of Wiskott Aldrich syndrome
Thrombocytopenia eczema recurrent infections
27
Which deficiency causes hyper IgM syndrome
CD40 ligand CD154 deficiency
28
Late complement deficiency predisposes to which infection
Neisseria infections
29
Early complement deficiency is associated with what disease
SLE like autoimmune disease
30
Which cancer risk is increased in CVID
Lymphoma and gastric carcinoma
31
Why is SCID a medical emergency
High risk of fatal infections in infancy
32
What is the main drug used for rheumatoid arthritis
Methotrexate
33
What is characteristic of Hyper IgE syndrome
High IgE coarse facies retained primary teeth
34
Name two drugs that cause secondary immunosuppression
Phenytoin and Captopril
35
Why are T cell defects called combined immunodeficiencies
B cells depend on T cell help
36
A 6-month-old boy presents with recurrent ear infections pneumonia and absent tonsils on examination Lab results show very low IgG IgA and IgM levels Flow cytometry reveals absent CD19+ B cells What is the most likely diagnosis? A. Severe Combined Immunodeficiency B. X-linked agammaglobulinemia C. Common Variable Immunodeficiency D. Selective IgA deficiency
B. X-linked agammaglobulinemia Explanation: Classic X-linked agammaglobulinemia — absent B cells + symptoms after maternal IgG declines (~6 months).
37
A child with recurrent catalase-positive infections forms granulomas in multiple organs Which mechanism is impaired? A. Antibody production B. T-cell activation C. Respiratory burst in phagocytes D. Complement activation
C. Respiratory burst in phagocytes Explanation: Chronic Granulomatous Disease → defective NADPH oxidase → no ROS → impaired intracellular killing.
38
A neonate develops severe infections within the first 48 hours of life Explain why this is unlikely due to a T-cell defect
Adaptive immunity (T and B cells) takes several days to activate Rapid onset suggests an innate immune defect (e.g. neutrophils or complement).
39
A child presents with eczema recurrent infections and thrombocytopenia He responds poorly to protein vaccines but relatively normally to polysaccharide antigens What is the underlying defect? A. B-cell maturation failure B. Complement deficiency C. Impaired T-cell polarization D. Neutrophil adhesion defect
C. Impaired T-cell polarization Explanation: Wiskott-Aldrich syndrome → defective T-cell function → poor protein antigen response.
40
A patient with recurrent Neisseria meningitis most likely has a defect in early complement components (C1–C4)
False Explanation: Late complement deficiency (C5–C9) → failure of MAC formation.
41
A patient with SCID receives a live vaccine and develops severe disseminated infection Why? A. Overactive innate immunity B. Lack of antibody production only C. Absence of both T and B cell function D. Excess cytokine release
C. Absence of both T and B cell function Explanation: Severe Combined Immunodeficiency → no adaptive immunity → cannot control even attenuated pathogens.
42
Delayed separation of the umbilical cord is a hallmark of ______
Leukocyte Adhesion Deficiency
43
A patient has recurrent infections with encapsulated bacteria but normal total immunoglobulin levels What is the most likely diagnosis? A. X-linked agammaglobulinemia B. Selective IgA deficiency C. Specific antibody deficiency D. SCID
C. Specific antibody deficiency Explanation: Failure to respond to polysaccharide antigens despite normal Ig levels.
44
Explain why patients with T-cell deficiencies often also have impaired antibody responses
B-cell activation (especially class switching and memory formation) requires helper T-cell signals (CD4+) so T-cell defects → combined immunodeficiency.
45
A patient with chronic renal failure develops widespread calcifications in normal tissues What is the mechanism? A. Local tissue necrosis B. Hypercalcemia due to secondary hyperparathyroidism C. Excess collagen deposition D. Lipid accumulation
B. Hypercalcemia due to secondary hyperparathyroidism Explanation: Metastatic calcification due to systemic calcium imbalance.
46
Which disease is caused by NADPH oxidase deficiency
Chronic Granulomatous Disease
47
Which condition is due to CD40 ligand deficiency
Hyper IgM syndrome
48
Which disease involves defective leukocyte adhesion
Leukocyte Adhesion Deficiency
49
A 4-year-old child presents with recurrent sinopulmonary infections Lab tests show low IgA but normal IgG and IgM What is the diagnosis? A. CVID B. Selective IgA deficiency C. SCID D. XLA
B. Selective IgA deficiency Explanation: Most common primary immunodeficiency.
50
Secondary immunodeficiencies are more common than primary immunodeficiencies
t
51
Why is SCID considered a pediatric emergency? A. Causes autoimmune disease B. Leads to malignancy C. Fatal infections occur early without treatment D. Causes only mild infections
C. Fatal infections occur early without treatment
52
Why do corticosteroids increase infection risk
They suppress immune responses (↓ cytokines ↓ T-cell activation ↓ inflammation) creating a prolonged immunosuppressed window.
53
A 6-month-old boy with recurrent infections and absent B cells most likely has: A. SCID B. CVID C. XLA D. IgA deficiency
C
54
Defect in NADPH oxidase causes: A. SCID B. CGD C. LAD D. CVID
B
55
Recurrent Neisseria infections suggest deficiency of: A. C1–C4 B. C3 C. C5–C9 D. IgA
C
56
Delayed umbilical cord separation indicates: A. CGD B. LAD C. SCID D. XLA
B
57
Poor response to protein vaccines suggests defect in: A. B cells only B. T cells C. Complement D. Neutrophils
B
58
SCID primarily affects: A. B cells only B. T cells only C. Both T and B cells D. Neutrophils
C
59
Most common primary immunodeficiency: A. CVID B. SCID C. IgA deficiency D. CGD
C
60
Hyper IgM syndrome is due to defect in: A. IgA B. CD40L C. C3 D. IL-2
B
61
Granuloma formation suggests: A. Complement deficiency B. CGD C. IgA deficiency D. CVID
B
62
Maternal IgG protects infants until: A. Birth B. 1 month C. 4–6 months D. 2 years
C
63
Rapid infection within 2 days suggests defect in: A. Adaptive immunity B. Innate immunity C. Memory cells D. Plasma cells
B
64
Failure of macrophage activation occurs with deficiency of: A. IFN-γ B. IL-10 C. IgG D. C5
A
65
CVID is associated with increased risk of: A. Diabetes B. Cancer C. Asthma D. Hypertension
B
66
Encapsulated bacteria infections suggest problem with: A. T cells B. Antibodies C. Platelets D. RBCs
B
67
LAD is due to defect in: A. Antibody production B. Leukocyte adhesion C. Complement D. Cytokines
B
68
SCID patients should avoid: A. Antibiotics B. Vaccines C. Live vaccines D. Steroids
C
69
Selective IgA deficiency affects: A. Blood B. Mucosal immunity C. Bone marrow D. Brain
B
70
Which cell is defective in CGD: A. B cell B. T cell C. Neutrophil D. RBC
C
71
Autoimmune disease is linked to deficiency of: A. Late complement B. Early complement C. IgM D. Platelets
B
72
Thrombocytopenia + eczema + infections = A. SCID B. Wiskott-Aldrich C. XLA D. CVID
B
73
SPUR stands for: A. Severe Persistent Unusual Recurrent B. Simple Passive Urgent Response C. Specific Primary Unknown Reaction D. Severe Primary Unusual Reaction
A
74
Which is NOT intracellular killing mechanism: A. ROS B. Enzymes C. Antibodies D. Nitric oxide
C
75
Complement MAC is formed by: A. C1–C3 B. C3–C5 C. C5–C9 D. C2–C4
C
76
Which immunodeficiency presents earliest: A. XLA B. CVID C. SCID D. IgA deficiency
C
77
Failure of class switching leads to: A. IgA deficiency B. Hyper IgM C. SCID D. LAD
B
78
Secondary immunodeficiency is more common
t
79
IgA deficiency causes severe systemic infections
80
SCID affects only T cells
81
CGD causes defective killing of bacteria
t
82
Complement deficiency affects innate immunity
t
83
XLA affects females
84
CVID presents in adulthood
t
85
LAD affects neutrophil migration
t
86
Hyper IgM has high IgG
87
T cells help B cells
t
88
SCID = Severe Combined ______ Deficiency
Immunodeficiency
89
CGD involves defective ______ burst
Respiratory
90
LAD affects leukocyte ______
Adhesion
91
IgA is important in ______ immunity
Mucosal
92
AIDS defined by CD4 count below ______
200
93
XLA caused by mutation in ______ kinase
Bruton
94
Hyper IgM due to defect in ______ ligand
CD40
95
Why does XLA present after 6 months?
Maternal IgG declines → no antibody protection
96
Why do T-cell defects affect B cells?
B cells need T-helper signals for activation and class switching
97
Why are SCID patients at high risk of infection?
No functional adaptive immunity (T + B cells)
98
Why does CGD cause granulomas?
Inability to kill microbes → chronic inflammation
99
Why do corticosteroids increase infection risk?
Suppress immune responses and inflammation
100
NADPH oxidase deficiency → ?
CGD
101
CD40L deficiency → ?
Hyper IgM syndrome
102
Selectin receptor defect → ?
LAD
103
MCQ - What is the fundamental underlying mechanism of autoimmune disease? Options: A. Excessive production of neutrophils in the bone marrow B. Defective elimination and/or control of self-reactive lymphocytes C. Acute infection by a single pathogen D. Overactive innate physical barriers
B. Defective elimination and/or control of self-reactive lymphocytes
104
Fill in the blank - Autoimmune disease is caused by an imbalance between ______ and ______ immune responses.
effector; regulatory
105
MRQ - Which of the following are reasons why autoimmune diseases are considered a significant clinical problem? Options: A. Their chronic nature B. High prevalence in people during prime working and reproductive years C. Associated high healthcare costs D. They only affect the central nervous system
A, B, C
106
FFQ - Describe the phases of autoimmune disease progression and how they might manifest clinically over time.
Autoimmune disease develops in three phases: initiation, propagation, and resolution. Clinically, it often shows a relapsing-remitting pattern, with phases of inactivity (remission) and exacerbation (symptomatic flares).
107
MCQ - Which factor contributes to autoimmunity by decreasing the efficiency of thymic selection and the function of Regulatory T cells (Tregs)? Options: A. Stress B. Aging C. Pesticides D. Structural modification of antigens
B. Aging
108
Fill in the blank - The release of "hidden" auto-antigens from privileged sites such as the ______ or ______ during trauma can trigger an autoimmune response.
CNS (Central Nervous System); lens (or spermatozoa)
109
MRQ - Based on the incidence chart provided in the sources, which of the following diseases show a significantly higher incidence in females than males? Options: A. Sjögren’s Syndrome B. SLE (Lupus) C. Myocarditis D. Type 1 Diabetes Mellitus
A, B
110
MCQ - Which HLA type is strongly associated with Ankylosing Spondylitis? Options: A. DRB103B. DQB102/08 C. B27D. C06:02
C. B*27
111
Fill in the blank - Coeliac disease is strongly associated with the HLA types ______ and ______.
DQB102; DQB108
112
MRQ - Which HLA alleles are associated with Type 1 Diabetes? Options: A. DRB103B. DRB104 C. B27D. C06:02
A, B
113
FFQ - Explain the role of genetic factors and environmental exposure in the development of autoimmunity.
Genetic susceptibility (polymorphisms in immune-regulating genes) creates a failure of self-tolerance, leading to the presence of self-reactive lymphocytes. Environmental stimuli (infection, tissue injury, UV) then activate tissue APCs, which in turn activate those self-reactive lymphocytes, leading to tissue injury and autoimmune disease.
114
MCQ - Where does central tolerance for T cells primarily occur? Options: A. Bone marrow B. Spleen C. Thymus D. Lymph nodes
C. Thymus
115
Fill in the blank - During T cell development in the thymus, the elimination of self-reactive T cells is known as ______ ______.
negative selection (or clonal deletion)
116
MRQ - Which of the following are mechanisms of peripheral tolerance? Options: A. Positive selection in the thymus B. Clonal anergy C. Deletion via apoptosis D. Suppression via Regulatory T cells (Tregs)
B, C, D
117
FFQ - Define "Epitope Spreading" and explain its clinical significance.
Epitope spreading occurs when tissue damage and alterations in self-proteins create new antigenic epitopes. These new epitopes activate more lymphocytes and recruit more immune cells, leading to more tissue damage and creating a vicious circle of spreading autoimmunity.
118
MCQ - What happens to a thymocyte (immature T cell) that recognizes self-MHC but interacts too strongly with a self-peptide during development? Options: A. It is signaled for survival (Positive selection) B. It is released into the circulation C. It undergoes negative selection (Signaled death/Apoptosis) D. It becomes a B cell
C. It undergoes negative selection (Signaled death/Apoptosis)
119
Fill in the blank - B cell central tolerance occurs in the ______ ______.
bone marrow
120
MCQ - In the germinal center, activated B cells undergo ______ ______ to improve their antibody affinity, which may inadvertently create new self-reactivity. Options: A. Clonal anergy B. Somatic hypermutation C. Positive selection D. Epitope spreading
B. Somatic hypermutation
121
MRQ - What factors help maintain peripheral tolerance in circulating T-lymphocytes? Options: A. Lack of HLA class II expression by normal tissue cells B. Inability of tissue cells to express co-stimulatory molecules C. High levels of TNF-alpha D. Suppression by Regulatory T cells (Treg cells)
A, B, D
122
FFQ - How does the body control a hypermutated B cell that acquires affinity for a self-antigen in the germinal center?
If a hypermutated self-reactive B cell encounters strong cross-linking of its B-cell receptor (BCR) by a self-antigen in the germinal center, it is induced to undergo apoptosis to prevent its release as an auto-reactive cell.
123
MCQ - What is the key transcription factor marker for Regulatory T cells (Tregs)? Options: A. T-bet B. FOXP3 C. GATA3 D. NF-kappaB
B. FOXP3
124
Fill in the blank - ______ Tregs develop in the thymus, while ______ Tregs arise in the periphery from naive CD4+ T cells.
Natural (nTregs); Induced (iTregs)
125
MRQ - Which of the following are mechanisms used by Tregs to suppress immune responses? Options: A. Secretion of IL-10 and TGF-beta B. Expression of CTLA-4 to inhibit co-stimulation C. Secretion of TNF-alpha D. Metabolic disruption or direct killing of effector cells
A, B, D
126
FFQ - Describe the clinical relevance of Treg dysfunction.
Dysfunction of Regulatory T cells leads to a loss of tolerance and the development of autoimmunity. Conversely, therapies involving Tregs are being investigated for treating autoimmune diseases and improving transplant tolerance.
127
MCQ - Which monogenic autoimmune disease is caused by a defect in the FOXP3 gene? Options: A. APS-1 B. ALPS C. IPEX D. Infantile enterocolitis
C. IPEX
128
Fill in the blank - Mutations in the AIRE gene lead to ______ ______ syndrome (APS-1) due to defective negative selection in the thymus.
autoimmune polyglandular
129
MRQ - Which genes are associated with Systemic Lupus Erythematosus (SLE) according to the "Genetically complex" table? Options: A. C2 and C4 (Complement proteins) B. FCGRIIB (Fc-gamma receptor) C. NOD2 D. CTLA4
A, B
130
MCQ - Defective apoptosis of self-reactive T and B cells in the periphery is the mechanism for which Mendelian disease? Options: A. IPEX B. ALPS (Autoimmune lymphoproliferative syndrome) C. APS-1 D. RA
B. ALPS (Autoimmune lymphoproliferative syndrome)
131
Fill in the blank - The theoretical possibility that sequence similarities between foreign and self-peptides result in cross-activation of autoreactive cells is called ______ ______.
molecular mimicry
132
MRQ - Which environmental factors are listed as potential predisposing triggers for autoimmunity? Options: A. UV radiation B. Vitamin D deficiency C. Chemicals such as smoking and pesticides D. Drug-induced reactions
A, B, C, D
133
FFQ - How does UV radiation contribute to the initiation of autoimmune diseases like SLE?
UV radiation induces apoptosis in many cells. This increases the burden of nuclear antigens (like DNA and chromatin) that are not normally exposed to the immune system, providing targets for autoantibodies.
134
MCQ - Which virus is associated with Rheumatoid Arthritis (RA), Autoimmune Hepatitis (AIH), and Thyroid disease? Options: A. Parvovirus B. Epstein-Barr virus (EBV) C. Coxsackie virus D. HCV
B. Epstein-Barr virus (EBV)
135
Fill in the blank - The bacterium ______ ______ is strongly linked to the development of Rheumatoid Arthritis (RA).
Porphyromonas gingivalis (or Campylobacter jejuni, etc.)
136
MRQ - Which of the following bacteria are listed in the sources as triggers for Rheumatoid Arthritis? Options: A. Streptococcus pyogenes B. Chlamydia C. Borrelia burgdorferi D. Salmonella typhi
A, B, C, D
137
FFQ - Describe the "Propagation" phase of autoimmune disease progression.
During propagation, physical symptoms become evident. This phase is characterized by a cytokine storm, elevated inflammatory markers like ESR and CRP, and a significant imbalance between T effector cells and Tregs. Stress or other illnesses can exacerbate this phase.
138
MCQ - What is the mechanism of Graves' Disease? Options: A. Autoreactive T cells against joint synovium B. Autoantibodies against the thyroid-stimulating-hormone (TSH) receptor C. Destruction of myelin sheaths by T cells D. Destruction of pancreatic islet beta cells
B. Autoantibodies against the thyroid-stimulating-hormone (TSH) receptor
139
Fill in the blank - Hashimoto’s thyroiditis results in ______ because of the destruction of thyroid tissue, leading to underproduction of thyroid hormones.
hypothyroidism
140
MRQ - Which autoimmune diseases primarily involve autoreactive T cells as the main mechanism of tissue destruction? Options: A. Type 1 Diabetes B. Multiple Sclerosis C. Graves' Disease D. Rheumatoid Arthritis
A, B, D
141
MCQ - Systemic Lupus Erythematosus (SLE) involves autoantibodies and T cells against which antigens? Options: A. Thyroid hormones B. Joint synovium C. DNA, chromatin, and ribonucleoprotein D. Myelin
C. DNA, chromatin, and ribonucleoprotein
142
Fill in the blank - Sjögren’s syndrome is characterized by lymphocyte infiltration of exocrine glands, leading most commonly to ______ ______ and/or ______.
dry eyes; mouth
143
FFQ - Why is it often difficult to pinpoint the exact factors responsible for the initiation of an autoimmune disease?
This is because patients usually only develop physical symptoms long after the abnormal immune reaction has already begun subclinically, making the original triggering event hard to identify in retrospect.
144
MCQ - What is a major limitation of most current autoimmune therapies? Options: A. They are too expensive for most patients B. They target the terminal phase of inflammation rather than the cause of the disease C. They only work in males D. They require monthly blood transfusions
B. They target the terminal phase of inflammation rather than the cause of the disease
145
Fill in the blank - Continuous, lifelong therapy for autoimmunity can result in an increase in ______ and ______ complications.
malignant; infectious
146
MRQ - Which of the following are listed as treatment options for autoimmune diseases? Options: A. Steroids for general inflammation suppression B. Cytokine antagonists like TNF-alpha blockers C. Monoclonal antibody therapy D. Antibiotics to cure the underlying cause
A, B, C
147
FFQ - Summarize the step-wise approach to treatment for autoimmune diseases.
Treatment is a step-wise process that should be reassessed at every stage. It generally moves from general inflammation suppression (steroids) to more targeted therapies like cytokine antagonists or monoclonal antibodies, often requiring lifelong management.
B4
flashcards
Decks in class (33)
# Cards
-
AP1: CD50
-
AP2: CA40
-
AP3: CD40
-
AP4: Inf40
-
AP5: C100
-
AP6: R60
-
AP7: CAL40
-
AP8: A40
-
I1: I116
-
I2: OTL50
-
I3: Inn40
-
I4: TC47
-
I5: BC40
-
I6: I45
-
I7: C50
-
I8: ID147
-
I9: HL50
-
I10: A93
-
I11: Hy138
-
H1: Ly45
-
H2: Li45
-
H3: K45
-
H4: Lu40
-
H5: S54
-
A1: L40
-
A2: UA50
-
A3: T50
-
A4: A50
-
A5: LL40
-
A6: P40
-
A7: HN60
-
AP9: T148
-
AP10: ST92
