Exam 3 Flashcards
Fungal and viral infections are mediated by what type of immunity
Cell mediated
- Tcell
- B-cell
- NKC
No Abs
What it is lacking is essential for metabolic fxn of TT-cellls and if absent leads to toxin for lymphocytes
SCID
Adenosine deaminase deficiency
Converts deoxyadenosine to deoxyinosine which isnt harmful
- T-cell
-Bcell
+ NKC
No abs
SCID
Part of VDJ recombo affecting receptor production
Associates with omen syndrome (its partial version)
Rag1/2
No BCR/TCR formation
- TCR
-BCR
+ NKC
No ABs
No repair of double stranded breaks (such as those created by VDJ recombo) - associates with radiosensitivity
SCID
Increased risk of developing lymphoma
Artemis
- T-cells
- NKC
+ B-cells
IgM only
SCID
Deficient in signal creation for receptors, cytoplasmic common gamma chain
Jak3 Deficiency
- T-cells
+ B-cells
+ NKC
IgM only
SCID
Lack of ability to stimulate differentiation of lymphoid progenitors into T-cells
IL-7R alpha chain deficiency
- T-cells
+ B-cells
+ NKC
IgM only
SCID
Lack of ability to recieve survival signals
CD3 complex deficiency
T-cell lymphopenia and inability to reject Severe opportunistic infections Chronic diarrhea No memory B-cells Symptoms once maternal IgG is depleted Thrush/candiditis Pneumocystis jiroveci No IgA for mucosal protection
HSTR and avoid live vaccines
SCID - severe combined immunodeficiency disorders
Agammaglobinulemia
Can still eliminate extracellular pathogens and bacteria but intracellular are impacted
Susceptibility to recurrent infections by encapsulated bacteria
Selective B-cell immunodeficiencies
-B-cell
+ T-cell
+ NKC
No Abs
B-cell defect
X-linked recessive
Defect in lg chain heavy chain rearrangement with main absence of IgG once child past 6 months
Agammaglobinulemia - X-linked BTK kinase deficiency
-T-cell
- NKC
+ B-cell
SCID- only one that is x-linked recessive
IgM only
Cell’s can’t be active to proliferate and used in many cytokines
MOST COMMON SCID
Common gamma chain deficiency
+ B-cell, T-cell, NKC
Can induce anaphylaxis due to transfusions
Asymptomatic but can develop autoimunity and allergies
Symptomatic treatment with no vaccine restrictions
IgA deficiency
+ B-cell, T-cell, NKC
Mostly normal abs
Defects in several genes with poor responses to polysacharides
Asymptomatic but recurrent viral/bacterial infections of respiratory tract
Symptomatic treatment with no restrictions for vaccines
IgG subclass deficiency
+ B-cell, T-cell, NKC
No class switching due ot lack of signal/somatic hypermutation
Loss of CD40L in T-cells, loss of CD40 in B-cells
Abnormal infections with encapsulated bacteria with ineffective protection/opsonization
Symptomatic - no polio
Hyper IgM
- B-cells low
+ T-cells
+ NKC
Infants create class switching later and have no humoral immunity for 3 years
Recurrent respiratory infections with susceptibilty to sinopulmonary infections
Symptomatic no polio
Transient hypogammaglobinuremia of infancy
Deficiency in CD3 circulating cells inducing opportunistic infections
Can induce autoimmunity or lymphoid malignancies
T-cell immyune deficiencies
+ B-cells
+ T-cellls
- NKC
Abs present
Lack of MHC II and therefore CD4
CD8 is intactt
SCID llike infections (recurrent respiratory, GI, urinary)
BLS II - bare lymphocyte syndrome
- T-cells
+ B-cells
+ NKC
Normal abs
Cardiac malformations with hypothyroidism and hypocalcemia
Frequent URIs
DiGeorge Syndrome (obviously thymus too)
+ B-cells
+ T-cells (low CD8 only)
- NKC
Normal abs
Tap 1 defect - transports peptide to ER resulting in inability to load
Recurrent viral infections due to CD8
MHC I deficiency
Defect in CD8 affecting integrin/sialyl lewis rolling
Phagocytes trap in circulation due to inability to migrate
Recurrent bacterial infections on skin/mucosal membranes
Neutrophilia in blood inducing lack of pus
Delayed detachment of umbilical, slow healing, death in early weeks
Phagocytic immunodeficiency
Leukocyte adhesion deficiency
X-linked
Deficient NADPH oxidase and failure to generate ROS impairing killing of extracellular pathogens like bacteria/fungi
Formation of granulomas
Susceptibiity to recurrent staph infections
Phagocytic immunodeficiency
Chronic granulomatous
X-linked hereditary
Inabillity to make NADPH resulting in loss of ROS
Formation of granulomas, ANEMIA DUE TO RBC METAB j
Asymptomatic
Cellular stressed/increased oxygen demand
Phagocytic immunodeficiency
Glucose-6-phosphate deficiency
deficient in enzyme that catalyzes conversion of hydrogen peroxide to hypthalus acid (bleach) - gives pus green color
Diabetic patient sduring infections and linked to neutrophil killing yeast
Immunohistologica staining of neturophils
Phagocytic immunodeficiency
Myeloperoxidase deficiency
Abnormal structure of neutrophils with giant granules that don’t contain cathepsin/elastace
Abnormalities in chemotaxis/degranulation/blunted neutrophil rexn
Albinism/recurrent infections due to staph/srept
Splenomegaly/lymphadenopathy
No NKC activity
Phagocytic immunodeficiency
Chediak Higashi syndrome - wheelchair bound too
Blood levels of proinflammatory cytokines are low due to impaird signaling
Frequent infections by pyogenic bacteria but normal resistance to other common bacteria, fungi, and viruses
Lack of fever
Increased susceptibility to herpes
Phagocytic immunodeficiency
TLR deficiency
Complement C that if lacking induces deposits in places such as kidneys - susceptibility to RA or SLE
C1/C4
Complement C that if deficient increases susceptibility to strept/sinopulmonary infections
C2
Complement deficiency with inability to activate C3 and associates with systemic maningococcal infections
Factor D
Fxns to activate factor B by cutting Bb in alternative path
Complement deficiency that functions to form Bb/Ba in alternative path and if missing systemic meningococcal infections
Factor B
Complement deficiency that is the base of kidney disease in hemolytic uremic syndrome via huge deposition of C3 in glomerular BM therefore constantly activating Alternatiive pathway
Functions to dissociate Bb from C3 normally
Factor H
Factor H/I deficiency
Damaged BM is unable to support complement activation and macular degneration occurs in kidney
factor I prevents continuous activation of classical pathway
Factor H inactivates AP by binding to C3b to displace Bb
Hemolytic uremic syndrome
C1-inhibitor deficiency
Leads to continuous activation of plasma complement
Body experiences sporadic inflammation in extremities due to overactive kallikrein-kinin systeme
Hereditary angioedema
C1-inhibitor inactivates kallikrein (kallikrein cleaves kininogen off to make bradykinin)
Bradykinin induces permeability in vessels
Lack of glycosylphosphatidylinisitol anchor in proteins
Lack of CD55 DAF: inhibts C3/C5 by dissociating either C2 or Bb
CD59: inhibits mac formation by blocking C9 polymerization
Protects RBC from susceptibility to complement and therefore if deficient they die
Paraxysmal nocturnal hemoglobinuria
Subcutaneous administration of a protein ag to immunized individual resulting in formation of complexes and vasculitis
Arthus rxn
Systemic vasculitis that affects arteries and induces aneurysms
Associates with hep B
Polyarteritis nodosa PAN
activating of TSH causing hyperthyroidism
Anxiety, irritability, tremor, and heat sensitivity
Graves
Inability to bind ach
Mystheria gravis
Type IV collagen destruction
Goodpastures
Cells associated with increased incidence of pyogenic bacteria
B-cell immunodeficiency
Defects in _ mediated immunity leads to infection by viruses and intracellular pathogens
T-cell (CD4)
This deficiency makes patients susceptible to pyogenic infections
X-linked hyper IgM syndrome
Defect in cell mediated immunity due to bad interaction for T-cel dependent activation of macrophages
Patients are susceptible to opportunistic intracellular microbes like pneumocytis jiroveci
Hyper IgM
Fungal suggests this type of cell defect
T
Another missing marker for LAD disease besides CD18 is
CD11
Recurrent bacterial infections that lead to stuff like otitis emdia and pneumonia are mediated by what cell immunity
B-cell immunity
