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primary immunodeficiency Flashcards

1
Q

common consequences of B cell deficiencies? what causes them?

A

bacterial and viral infections

absent or reduced follicles and germinal centers
reduced IG

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2
Q

common consequences of T cell deficiencies? what causes them?

A

Viral and Intracellular infections, cancers

some cancers

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3
Q

common consequences of innate immune deficiencies? what causes them?

A

viral and bacterial infections

deficiencies in innate immunity

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4
Q

infections due to immunodeficiencies?

when can they arise and what are the symptoms?

A

may arise at ANY AGE

-they are often chronic, severe, or recurrent
- they are not responsive well to an ANTIBIOTIC THERAPY
- MICROBES involved may be atypical which normally do
not often seen in clinical practice
-OPPORTUNISTIC infections!!

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5
Q

OPPORTUNISTIC ORGANISMS? significance?

A

pathogens have low virulence

Opportunistic infections occur when host defenses are
immunocompromised

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6
Q

Primary Immune

Deficiencies? when do they come and why?

A

clinically manifested during the FIRST YEARS OF LIFE when maternal IgGdisappear after 6 months. Prior to that moment, PIDs usually are not detected in
the NEWBORN

Newborn and infants with PIDs suffer from RECURRENT and PROTRACTED
INFECTIONS which leads to diagnosis of PIDs

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7
Q

What are PID? what causes them?

A

-Defects in innate immunity
» Deficiencies of phagocytosis
» Deficiencies of complement

– Defects in adaptive immunity
» Antibody deficiencies (B cells)
» Deficiencies of T-cells
» Combined T- and B-cell deficiencies

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8
Q

what are the signs of IDs?

A

Eight or more ear infections in one year.
• Two or more serious sinus infections in one year.
• Two or more bouts of pneumonia in one year.
• Two or more deep-seated infections, or infections in unusual areas.
• Recurrent deep skin or organ abscesses.
• Need for iv antibiotic therapy to clear infection.
• Infections with unusual or opportunistic organisms.
• Family history of primary immunodeficiency.

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9
Q

Suspected PID? what do you do?

A

-Serum IgG, IgM, and IgA, Ab testing to specific Ag
after immunization: Screen humoral immunity

-Differential count of blood
cells, DTH skin test: Screen cellular
immunity

-Nitroblue tetrazolium test: Screen for phagocyte
defect

-Total hemolytic complement
assay: Screen for complement
deficiency

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10
Q

What factors lead to VDJ recombination?

A

RAG1, RAG2, Artemis

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11
Q

Pre BCR to immature B? what do we need?

A

RTK

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12
Q

How are CD8 T cells made?

A

Zap70, Tap 1 and 2

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13
Q

SEVERE COMBINED IMMUNODEFICIENCY? how is it diagnosed and reason?

A
  • 4–6 months of age with severe persistent
    infections, oral thrush, chronic diarrhea, and failure to thrive

-affected infants have a permanent profound T-cell
lymphopenia with or without reduced numbers of circulating B cells and natural killer (NK) cells

-T-cell mitogens are depressed and T-cell
receptor levels are low or undetectable

  • placed in protective isolation and offered hematopoietic stem cell
    transplantation
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14
Q

What Igs and cell components are low in SCID? what are the consequences?

A

IgG, IgM, and IgA

profound DEFICIENCIES of
T-cell and B-cell functions

at RISK of abortion due to INABILITY to reject the
maternal T cells

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15
Q

what does adenosine deaminase do?

A 
converts toxic for
lymphocytes
deoxyadenosine into
deoxyinosine, which is
not harmful.
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16
Q

what does artemis do?

A 
enzyme in VDJ
recombination and
serves to repair
double strand
breaks.
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17
Q

what is it called? what happens

adenosine deaminase deficiency

A

t-b-nk-?
Low IgG, IgA, and IgM
AUTOSOMAL RECESSIVE disorder, most common cause of scid

ADA deficiency leads to an ACCUMULATION of toxic for lymphocytes
metabolic by-products deoxyadenosine

ADA is essential for the METABOLIC FUNCTION of various cells,
especially T-cells

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18
Q

t-b-nk+? what is it called?

A

artemis deficiency

rag1rag2 deficiency

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19
Q

what is called and what happens?

Purine Nucleoside Phosphorylase (PNP) Deficiency

A

t-b+nk+/-

Normal IgM, IgG, and IgA
ACCUMULATION of intracellular deoxyguanosine
triphosphate (dGTP). This metabolite is toxic to lymphocytes, leading to a
DECREASE in peripheral T cell numbers.

EARLY ONSET NEUROLOGICAL ABNORMALITIES

AUTOIMMUNE DISORDERS are also common and include hemolytic anemia,
thyroid disease, arthritis, lupus

HSCT is the definitive TREATMENT for PNP deficiency patients

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20
Q

Artemis Deficiency? what is it called and what happens?

A

T-,B-,NK+
Low IgG, IgA, and IgM

AUTOSOMAL
RECESSIVE RADIOSENSITIVE SCID: Native Americans!

diarrhea, candidiasis, and
Pneumocystis jiroveci fungus, radiosensitivity!

increased risk of developing
lymphomas

HSCT

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21
Q

RAG1/RAG2

Deficiency? what is it called and what happens?

A

T-,B-,NK+
Low IgG, IgA, and IgM

AUTOSOMAL RECESSIVE
disorder causing SCID

IMPAIRED V(D)J RECOMBINATION and
this leads to defective expression of the pre-TCR and pre-BCR

diarrhea, candidiasis, and
Pneumocystis jiroveci pneumonia.

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22
Q

Omenn syndrome?

A

Leaky RAG1/RAG2 defects allow for partial function of RAG1/RAG2
can give rise to an atypical form of SCID

evere erythroderma,
splenomegaly, eosinophilia, and high IgE

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23
Q

Deficiency of Jak3? what happens what does it do?

A

T-,B+,NK+
Very low IgG, IgA, and IgM

MUTATION in a gene that encodes a
lymphocyte Janus kinase 3 (Jak3)

AUTOSOMAL RECESSIVE trait
BOYS and GIRLS can be equally affected

Causes DEFECT in IL-2 receptor signaling

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24
Q

what is the relevance of antibody deficiencies ?

A

most common immune deficiency

state in infants and children, accounting for nearly half of all conditions

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25
Agammaglobulinemia? what is it and what does it do?
inherited as an X-linked trait, but autosomal recessive (AR) forms also exist B-CELL DEVELOPMENT is arrested at the pre-B-cell stage circulating B cells are usually absent or present in very low numbers
26
X-linked Btk | Kinase Deficiency, what is it and what does it do?
B –, T + , NK + No IgG, IgM, IgA X-LINKED disorder DEFECT in rearrangement of the Ig heavy chain genes IgG, IgA, IgM are totally absent or very low 5-6-month old infants diagnosis
27
``` Isolated IgG Subclass Deficiencies, what is it and what does it do? ```
B –, T + , NK + Some IgG subclasses LOW; normal IgM, IgA, IgE decreased CONCENTRATIONS of one or more IgG subclass. DEFECTS in several genes usually asymptomatic but may be associated with RECURRENT VIRAL/BACTERIAL INFECTIONS, frequently involving the respiratory tract. Low levels of IgG2 are frequent associated with poor responses to polysaccharide Ags in children IgG4 levels vary widely and many healthy people have no IgG4
28
IgA Deficiency? what is it and what doe it do?
B +, T + , NK + No IgA; normal IgG & IgM higher in male patients often develop AUTOIMMUNE DISEASES and ALLERGY THE INCIDENCE is relatively high Most affected individuals HEALTHY. Multiple genes are involved IgA secreting B cells may have disorders of maturation or terminal differentiation titers of IgA is undetectable or very low, IgG and IgM are normal. In some patients, IgA is synthesized but not secreted Patients with undetectable IgA levels may have serum anti-IgA IgG linked to the development of non-IgE mediated ANAPHYLAXIS in response to an intravenous immunoglobulin (IVIG) transfusion
29
DiGeorge Syndrome? what is it and what does it do?
T -, B +, NK + Normal IgG, IgA, and IgM T-cell deficiency!! MICRODELETION of 22q11.2 region containing more than 35 genes THE CLASSIC TRIAD:cardiac anomalies, hypocalcemia, and hypoplastic thymus HUMORAL IMMUNITY is intact in most patients!!!! frequent upper respiratory infections LIVE VIRAL VACCINES are generally given to patients who have a CD8 T-cell count > 300 cells/mm3
30
Hyper IgM | Syndromes (HIGM), what are they and what do they do?
B +, T + , NK + High IgM; low IgG & IgA ``` impaired Ig class switching and somatic hypermutation ``` NORMAL numbers of peripheral B cells, but LOW numbers of CD27-positive memory B cells increased SUSCEPTIBILITY TO BACTERIAL INFECTION issue in CD40L gene!! binds CD40 expressed on B cells triggers terminal differentiation of B cells X-LINKED CD40L DEFICIENCY (male only) is responsible for 2/3 of all cases of HIGM AUTOSOMAL CD40 DEFICIENCY (female and male) deficiency accounts for 1/3 of cases of HIGM
31
Transient Hypogammaglobulinemia of Infancy, what is it and what does it do?
B +, T + , NK + Low IgG/IgA ; IgM normal or low Ig production is delayed for up to 36 months in infants low IgG and IgA concentrations, but IgM concentration may be NORMAL or LOW INCREASED SUSCEPTIBILITY to sinopulmonary infections. normalize between 2 and 4
32
Common Variable Immune | Deficiency (CVID), what is it and what does it do?
B –/+, T + , NK + LOW IgG and IgA; sometimes low IgM hetergenous DEFECTs in Ab production associated with HYPOGAMMAGLOBULINEMIA mutations in receptors for B cell growth factors (maturation/activation) and costimulators recurrent pyogenic sinopulmonary infections NUMBER of circulating B cells is reduced or normal. B cells fail to differentiate into plasma cells which secrete Abs CVID are at an INCREASED RISK of infections, autoimmune diseases, and malignancies AUTOSOMAL DISORDER - both males and females are equally affected
33
Humoral | Immunity Screen?
measure IG levels ``` QUANTITATIVE IMMUNOGLOBULIN LEVELS normal? -Consider complement, phagocytic defect, other conditions ``` QUANTITATIVE IMMUNOGLOBULIN LEVELS abnormal? -Screen cellular immunity ``` Screen cellular immunity abnormal? -Consider diagnosis of Common variable immunodeficiency (CVID) ``` Screen cellular immunity normal? - Consider diagnosis of: 1. X-linked agammaglobulinemia 2. Isolated IgA or IgG 3. Hyper IgM
34
T-CELL IMMUNODEFICIENCY manifestations?
extreme susceptibility to opportunistic infections or disorders featuring predominately autoimmunity or predisposition to cancer
35
Common g Chain Deficiency | (gc or IL-2Rg), what is it and what does it do?
Very low IgG, IgA, and IgM T -, B +, NK - MOST COMMON form of SCID X-LINKED RECESSIVE GAMMA-CHAIN shared by the T-cell growth factor receptor (IL-2Rg) and other growth factor receptors no functional B cells since T-cells unable to “help” failure to thrive, severe thrush, opportunistic infections, and chronic diarrhea
36
IL-7R Alpha Chain Deficiency? what is it and what happens?
T -, B +, NK + Very low IgG, IgA, and IgM AUTOSOMAL RECESSIVE IL-7 plays a KEY ROLE in early T cell development IMMUNOGLOBULIN are low to absent despite the presence of B cells due to absence of T cell co-stimulatory signaling candidiasis, chronic diarrhea, Pneumocystis jiroveci pneumonia, severe viral infections Sequencing of the IL-7R gene can confirm the diagnosis
37
Bare Lymphocyte | Syndrome Type 2 ? what is it and what does it do?
``` CD4 T -, CD8 T +, B +, NK - rare AUTOSOMAL RECESSIVE GENETIC disorder causing an HLA class IInegative SCID ``` ``` no MHC class II expression on professional APCs that causes a DEFICIENCY in CD4+ T cells ``` MUTATIONS are in genes which encode for transcription factors that normally regulate the expression of the MHC II genes MHC class II genes on chromosome 6 are intact Variable HYPOGAMMAGLOBULINAEMIA (mainly IgA and IgG2). RECURRENT respiratory, gastrointestinal, and urinary tracts infections and frequently to death in early childhood
38
MCH Class I Deficiency? what is it and what does it do?
TAP1 molecules to transfer peptides to ER CD8+ cells are deficient that causes recurring viral infections CD4+ cells are normal. • Normal Ab production . • Normal DTH
39
CD3 Complex | Deficiencies, what is it and what happens?
T -, B +, NK + Low IgG, IgA, and IgM AUTOSOMAL RECESSIVE form of SCID Deficiencies of the CD3 SUBUNITS (delta, gamma, epsilon, or zeta) PRESENTS IN INFANCY with lymphopenia and decreased T cell numbers. B cell and NK cell numbers are normal ANTIBODY RESPONSES are typically decreased failure to thrive, opportunistic infections, and chronic diarrhea HSCT is indicated for patients who have a severe T-B+NK+ SCID phenotype
40
Defect in the IFN-g–IL-12 AXIS? what is it and what happens?
POSITIVE REGULATORY LOOP IL-12 Produced by Mf and DCs binds to IL-12R stimulates IFN-g release by T cells and NK cells Binding of IFN-g by Mf crosslink the IFNgR and activate the production of H2O2 and TNF-a and IL-12 Mutations resulting in increased SUSCEPTIBILITY to nontuberculous mycobacteria have been identified in the genes encoding: – the IFN-g receptor – the IL-12 receptor – the p40 subunit of IL-12.
41
Defects in IL-12/IFN-g Pathway? what is it and what happens?
IL-12 signaling is ESSENTIAL FOR THE DIFFERENTIATION of naïve T cells into Th1 cells MUTATIONS in the IL-12 or IL-12R genes result in a clinically limited primary immunodeficiency Patients with IL-12 or IL-12R deficiency do not produce Th1 cytokine IFN-g which is necessary for the control of intracellular bacterial infections SELECTIVE SUSCEPTIBILITY to intracellular pathogens such as atypical mycobacteria also have defects in formation of IL-17 producing Th17 cell that accounts for the recurrent fungal infections
42
Th17 Deficiency, what is it and what happens?
UNUSUAL SUSCEPTIBILITY to chronic mucocutaneous candidiasis h MUTATIONS in genes encoding for either IL-17, IL-17R, or transcriptional factors STAT1, STAT3 or AIRE.
43
IPEG, what is it and what happens?
defect in tcell function ,SELF-REACTIVE T EFFECTOR CELLS ARE NOT INHIBITED, because of a mutation in the FOXP3 results in loss of inhibition by CD4+CD25+ Treg cell
44
ALPS, what is it and what happens?
defects in either Fas, FasL, caspase-8 or caspase-10 genes results in abrogated formation of the death-inducing signaling complex (DISC) and resistance of effector T cells to apoptosis.
45
Wiskott-Aldrich Syndrome (WAS), what is it and what happens?
T -, B +, NK - Low IgM; normal Ig, elevated IgA and IgE X-LINKED DISORDER MUTATIONS in the Wiskott-Aldrich Syndrome Protein (WASP) thrombocytopenia, eczema, cellular and humoral immunodeficiency, autoimmune disease, and malignancy COMBINED IMMUNODEFICIENCY -Decreased IgM, normal IgG, and elevated IgA and IgE levels – T cell lymphopenia – Decreased NK cell cytotoxicity RECURRENT BACTERIAL INFECTIONS with encapsulated bacteria
46
NK Cell Deficiency (NKD), what is it and what happens?
40 IMMUNODEFICIENCIES known to impair NK cells NK cells should represent the major immunologic abnormality in the patient MUTATIONS in multiple genes Classical NKD,Functional NKD multiple severe or disseminated viral infections
47
Classical NKD?
defined as an absence of NK cell
48
Functional NKD?
defined as the presence of NK cells | exhibiting defective NK cell activity without NK cell lymphopenia
49
Cellular | Immunity Screen? when do we do it
CBC/differential + chest X-ray – if no thymus – consider DiGeorge syndrome; SCID (especially Absolute Lymphocyte Count) If the tcell panel is abnormal -Functional testing -B cell/NK cell CD marker studies if those are abnormal: SCID – do DNA testing or specific enzyme defect Complete DiGeorge syndrome
50
Leukocyte adhesion deficiency: cellular abnormality? immune defect? associated infections?
defective CD18 defective migration of phags into tissues widespread infections with capsule bacteria
51
chronic granulomatous disease: 1. cellular abnormality? 2. immune defect? 3. associated infections?
1. defective NADPH oxidase 2. impaired killing of phagocytosed bacteria,FAIL to generate superoxide anion 3. chronic bacterial and fungal infections,susceptible to RECURRENT INFECTION with catalase positive organisms tendency to form granulomas MOST FREQUENT phagocytic primary immunodeficiency
52
G6PD deficiency 1. cellular abnormality? 2. immune defect? 3. associated infections?
1. G6PD deficiency, respiratory burst not working, substrate for NADPH not there 2. impaired killing of phagocytosed bacteria 3. chronic bacterial and fungal infections, anemia by certain agents X-LINKED RECESSIVe, associated with anemia, mostly asymptomatic
53
Myeloperoxidase deficiency cellular abnormality? immune defect? associated infections?
Myeloperoxidase deficienct in granules, production of toxic oxygen species impaired killing of phagocytosed bacteria chronic bacterial and fungal infections
54
Chediak Higashi syndrome? 1. cellular abnormality? 2. immune defect? 3. associated infections?
1. defect in vesicle fusion 2. impaired phagocytosis, endosome cant fuse with phagosome 3. recurrent and peristent bacterial infections, granulomas AUTOSOMAL RECESSIVE ABNORMALITIES in chemotaxis and degranulation GRANULES do NOT contain cathepsin G and elastase, abnormal giant granules PATIENTS become wheelchair-bound and usually die of infection in their early 30s
55
Leukocyte Adhesion Deficiency (LAD), what is it and what happens?
without the ongoing infection, NEUTROPHIL COUNT in the blood is about twice the normal level RECURRENT INFECTIONS of the oral and genital mucosa, skin, and intestinal and respiratory tracts NEUTROPHILS in patients with LAD are unable to aggregate defect in adhesion and targeting to sites of microbial invasion Neutrophils do not bind to INTERCELLULAR ADHESION molecules on endothelial cells INFECTED FOCI contain few neutrophils and heal slowly
56
Molecular defects in LADs result in what?
– recurrent infections with bacteria and fungi | – inability to form pus at extravascular sites
57
what is LAD1 caused by?
mutations in the gene for the b2 integrins (CD11/CD18), resulting in their decreased or absent levels on leukocytes
58
what is LAD2 caused by?
impaired adhesive functions of PSGL1 (P-selectin glycoprotein ligand 1) caused by MUTATIONS in a fucose transporter and consequent defective fucosylation of PSGL-1.
59
What is LAD3 caused by?
, there is DEFECTIVE activationdependent SIGNALING of b2 integrins resulting in adhesion defects similar to those in LAD-1.
60
What is LAD caused by? clinical manifestations and prognosis?
DEFECTIVE MIGRATION of leukocytes ``` manifestations?: – Delayed detachment of the umbilical cord – Slow wound healing – Severe bacterial infections – Failure to form pus ``` – the outcome is poor with EARLY DEATh
61
what does LAD workup involve?
FLOW CYTOMETRIC ASSESSMENT of the neutrophil | adhesion molecules CD11 and CD18
62
What is Chediak-Higashi caused by? how many phases? what is the diagnosis?
``` recurrent pyogenic GRANULOMAS caused by bacteria infections (staphylococci and streptococci) ``` blunted NEUTROPHILIA and due to delayed diapedesis ``` diphasic: » First phase – succesptibility to INFECTIONS. » Second phase – an accelerated LYMPHOPROLIFERATIVE SYNDROME with hepatosplenomegaly and lymphadenopathy ``` diagnosis: – azurophilic GIANT cytoplasmic INCLUSIONS in blood cells – Partial ALBINISM (defect in growth of melanocytes) – NO NK activity
63
Phagocyte Defect Screen? what does it do and what is it used for?
check for Chediak-Higashi syndrome Recurrent skin abscesses and/or fungal infections
64
What does C1, C4 and C2 manifest as?
lupus erythematosus-like AUTOIMMUNITY recurrent sinopulmonary infections
65
DEFECTS IN C3 manifest as?
INDISTINGUISHABLE from ANTIBODY DEFICIENCIES, although this complement deficiency is markedly less frequent than humoral ab-dependent immunodeficiencies
66
Defects in the LATE COMPONENTS of complement manifest as?
INCREASED SUSCEPTIBILITY to infections with Neisseria species
67
ALTERNATIVE COMPLEMENT pathway defects, what happens and how does it manifest?
-deficiencies Properdin, factor B and factor D cause infections caused by Neisseria meningitis and other extracellular bacteria -FACTOR H DEFICIENCY atypical hemolytic uremic syndrome or glomerulonephritis
68
C1 ESTERASE INHIBITOR what happens?
hereditary angioedema, complement issue RECURRENT SWELLING in the extremities, face, lips, larynx or GI tract involves not the complement enzymes, but a byproduct of the kinin-generating pathway production of BRADYKININ through this pathway that is responsible for the tissue permeability changes that cause the swelling Acute TREATMENTS include C1 inhibitor, a replacement therapy
69
DECAY-ACCELERATING FACTOR defects?
paroxysmal nocturnal hemoglobinuria
70
Primary C1 and C4 DEFICIENCY is linked to what?
development of systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA). -Small complexes are cleared from the circulation when they bind to complement receptors on RBCs -Without complement, the complexes can grow too large to be easily cleared -These large complexes are no longer soluble, and form deposits in the tissues and become a site of inflammation
71
C2 DEFICIENCY significance?
most common complement deficiency in Caucasian populations (1 in 10,000 to 1 in 20,000). – found in young children who have recurrent infections with Streptococcus pneumoniae
72
C8 Complement Deficiencies? significance, causes, symptoms
AUTOSOMAL RECESSIVE deficiencies of the late complement proteins INCREASED SUSCEPTIBILITY to Neisserial infections. THE CAUSE of low levels of C8 may: – inherited deficiencies – acquired deficiencies – due to complement consumption DIAGNOSIS: – Absent C8 levels in the presence of normal C3 and C4 values are consistent with a C8 deficiency. – Absent C8 levels in the presence of low C3 and C4 values suggests complement consumption.
73
What is HAE, what causes it and what happens?
painful, swelling recurrently in body C1 Inh not working properly -increases bradykinin, causing swelling 3 types of swelling: 1. peripheral 2. abdominal 3. episodes that make breathing difficult
74
Paroxysmal Nocturnal | Hemoglobinuria , what happens and why?
FAILURE to regulate the formation of the MAC. SOMATIC MUTATION causes a deficiency of glycosylphosphatidylinositol CD55 and CD59 are complement regulatory proteins involved in protecting the red blood cells from the action of complement CAUSE of intravascular hemolysis in these patients is the increased susceptibility of red cells to complement
75
what does CD59 do?
inhibits the | formation of the MAC by binding to sites on C8 and C9
76
what does DAF (CD55) do?
inhibits C3 and C5 convertases
77
what is TLR caused by and what happens?
MyD88 DEFICIENCY is an innate immune deficiency that results in impaired signaling for all TLRs but TLR3 frequent and SEVERE INFECTIONS caused by pyogenic bacteria NORMAL RESISTANCE to other common bacteria, viruses, fungi, and parasites characteristically lack FEVERS and elevated levels of ESR/CRP despite active infection levels of PROINFLAMMATORY TNF-a, IL-1, and IL-6 are LOW
78
what is the significance of TLR3 deficiency?
autosomal dominant disorder which results in INCREASED SUSCEPTIBILITY to HSV encephalitis

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