Immune DO Flashcards by MA CARMELA CONCEPCION SALES

Common Organisms for B cell deficiency

Recurrent bacterial: streptococci, staphylococci, Haemophilus, Campylobacter;

Viral:enteroviruses;

Uncommon: giardia, cryptosporidia

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Common Organisms for T cell deficiency

Opportunistic organisms: CMV, EBV, varicella, Candida,

Pneumocystis jiroveci, mycobacteriwa

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Common Organisms for Complement deficiency

Pneumococci, Neisseria

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Common Organisms for neutrophil deficiency

Bacteria: Staphylococci, Pseudomonas, Serratia, Klebsiella, Salmonella;

Fungi: Candida, Aspergillus

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Age onset

B-Cell

T-Cell

Complement

Neutrophil

5-7 months of age or later childhood to adult

Usually 2-6 months of age

Any age

Early onset

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Types of infections wth

B-Cell deficiency

Most are recurrent sinopulmonary infections and recurrent

enteroviral meningitis

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Types of infections wth

T Cell deficiency

Mucocutaneous candidiasis; pulmonary and GI infections

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Types of infections wth

Complement deficiency

Meningitis, arthritis, septicemia, recurrent sinopulmonary

infections

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Types of infections wth

Neutrophil deficiency

Skin abscesses, impetigo, cellulitis, suppurative adenitis,

gingivitis, oral ulcers, osteomyelitis, internal organ abscesses

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Other findings with:

B-Cell deficiency

Autoimmunity, lymphoreticular malignancy

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Other findings with:

T-Cell deficiency

Chronic diarrhea and failure-to-thrive;

postvaccination dissemination - varicella, BCG;

hypocalcemia in infancy;

graft-versushost from transplacental maternal engraftment or nonirradiated blood

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Other findings with:

Complement deficiency

Autoimmune disorders, vasculitis, glomerulonephritis,

angioedema

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Other findings with:

Neutrophil deficiency

Prolonged attachment of umbilical cord, poor wound healing, decreased signs of infection

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Best initial test

B-Cell deficiency

Screen with IgA→if low, measure IgG and IgM (quantitative

immunoglobulins

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Best initial test

T-Cell deficiency

Lymphocyte count (low)

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Best initial test complement deficiency

Screen is total hemolytic complement (CH50)—will be

depressed if any component is consumed

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Best initial test neutrophil deficiency

Neutrophil count

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Best cost-effective test for T-cell function –

Candida skin test

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Complemend deficiency

all are autosomal except for _______

properdin deficiency
(X-linked

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Neutrophil respiratory burst after phorbol ester stimulation;
most reliable now uses_________

rhodamine fluorescence (replaced the NBT test

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Specific test

B-Cell deficiency

Enumerate B-cells with flow cytometry (monoclonal antibodies to B-cell-specific CD antigens): B cell absent or
present and number

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Specific tests:

T cell deficiency

Flow cytometry using monoclonal antibodies recognizing
T-cell CD antigens (phytahemmaglutinin,
concanavalin A, pokeweed mitogen

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profound defect in B-cell development
which leads to an absence of circulating B cells and thus leads to severe hypogammaglobulinemia
with small-to-absent tonsils and no palpable lymph nodes

X-linked (Bruton) agammaglobulinemia

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> 500 known mutations of the _______ which is necessary for pre-B-cell expansion and maturation; long arm of X-chromosome

Btk gene (Bruton tyrosine kinase),

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Findings for X-linked (Bruton) agammaglobulinemia

boys with pyogenic sinopulmonary infections

X-linked (Bruton) agammaglobulinemia Tx

appropriate use of antibiotics + regular monthly IVIG

The only 2 B-cell defects for which stem cell transplantation is recommended are ________ and ______

CD40 ligand defect (extremely rare; one of the known mutations on the X-chromosome for hyper IGM syndrome) and X-linked lymphoproliferative disease

Common Variable Immunodeficiency (CVID) is _____________

hypogammaglobulinemia with phenotypically | normal B-cells;

What is the problem with CVID

Common Variable Immunodeficiency (CVID) is hypogammaglobulinemia with phenotypically normal B-cells;

CVID clinical presentation + serum IG and antibody deficiencies as profound or less than in XLA; normal sized lymphoid tissue; later autoimmune disease and malignancy __________

(lymphoma

Whast is the Tx of CVID

therapy consists of the one IG preparation available that contains no IgA.

__________ is the most common immunodeficiency. It is caused by the absence or near absence of serum and secretory IgA with phenotypically normal B-cells

Selective IgA deficiency

Selective IgA deficiency Clinical findings: same bacteria as others with most infections in respiratory, GI and urogenital tracts;_________ is common

giardiasis

serum antibodies to IgA can cause severe _______ if any blood product with IgA is administered (NOT a transfusion reaction)

anaphylactic reactions

Tx of IgA Def

Treatment: IVIG is not indicated (95−99% is IgG) because if usual IVIG (containing IgA) product is given, patients are at risk for severe reaction. Additionally, because it is specifically an IgA deficiency, the IVIG product with the IgA removed cannot be used. Treat the infections (generally milder).

________is thymic and parathyroid hypoplasia to aplasia from dysmorphogenesis of the 3rd and 4th pharyngeal pouches

DiGeorge syndrome

Other structures involved in Di Georg

great vessel anomalies (right-sided aortic arch, interrupted aortic arch), esophageal atresia, bifid uvula, congenital heart disease (conotruncal malformations, septal defects), facial dysmorphism (short philtrum, thin upper lip, hypertelorism, mandibular hypoplasia, low-set, often notched ears), and cleft palate

What is the CATCH 22 syndrome

Cardiac, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia); partial DiGeorge is more common, with variable thymic and parathyroid hypoplasia.

About 1/3 with complete DiGeorge have the ____

CHARGE association.

Initial presentation of Di George is?

neonatal hypocalcemic seizures

Tx of Di Georg

complete form correctable with either culture unrelated thymic tissue transplants or bone marrow or peripheral blood transplantation from HLA-identical sibling

_________ is the absence of all adaptive immune function, and in some, natural killer cells due to diverse mutations. It is the most severe immunodeficiency known

Severe Combined Immunodeficiency (SCID)

Clinical findings associated with SCID

first 1-3 months of life with recurrent/persistent diarrhea and opportunistic infections that may lead to death;

Pts with SCID also at risk for graft-versus-host disease from ________that crossed the placenta in utero

maternal immunocompetent T-cells

the presence of low but not absent T-cell function and low but not absent antibodies; patients survive longer but have failure-to-thrive and still die relatively early in life

Combined immunodeficiency

_________ is an impaired humoral immune response and highly variable concentrations of the IGs with moderately reduced T-cells and variable mitogen responses.

Wiskott-Aldrich Syndrome

Clinical findings of WAS

(1) thrombocytopenia presenting in neonatal period (2) atopic dermatitis, and (3) recurrent infections in first year of life

Dx of WAS

most common IG pattern is low IgM, | high IgA and IgE and normal to slightly low IgG and variably reduced T-cells.

WAS Rare survival beyond adolescence (bleeding, infections and EBV associated malignancies and autoimmune complications) without a _______

bone marrow | transplant

______ is a moderately depressed response to T and B-cell mitogens, moderately reduced CD3 and CD4 T-cells with normal or increased percentages of CD8, T-helper cell and intrinsic B-cell defects, and hypoplastic thymus

Ataxia-telangiectasia

Mutation of AT

AT mutation (ATM) at 11.22-23

AT (1) ataxia evident with onset of walking and progresses until age______ when confined to a wheelchair (2) ______ develop at 3-6 years of age and (3) recurrent sinopulmonary infections most with common viruses and occasional fatal _____

10-12 years oculocutaneous telangiectasias varicella

___________ is a rare disorder of leukocyte function causing recurrent bacterial and fungal infections and decreased inflammatory responses in the presence of neutrophilia (increased counts

Leukocyte adhesion deficiency

MC infections associated with Leukocyte Adhesion Deficiency

most common organisms are S. aureus, gram-negatives and Candida and Aspergillus

Leukocyte Adhesion Deficiency Dx

paucity of neutrophils in affected tissue but circulating neutrophil count is significantly elevated

Tx of Leukocyte Adhesion Def

early allogenic stem-cell transplantation for severe forms otherwise supportive care

_________is when neutrophils and monocytes phagocytize but cannot kill catalase-positive microorganisms as a result of a defect in production of oxidative metabolites

Chronic granulomatous disease (CGD)

* Autosomal recessive | * Abnormal secretory/storage granules lead to large and irregular seen in neutrophils

Chediak-Higashi Syndrome

Associations of Chediak-Higashi Syndrome

Oculocutaneous albinism from birth, prolonged bleeding time, peripheral neuropathy, recurrent infections

All components are autosomal recessive or co-dominant, except for ______ which is X-linked recessive

properdin deficiency

Decrease in both _____ and ____suggests activation of the alternative pathway; this is most useful in distinguishing nephritis secondary to immune complex deposition from that due to nephritic factor

C3 and C4

Results of Defect in complement function

recurrent angioedema, autoimmune disease, chronic nephritis, HUS, recurrent pyogenic infections, disseminated meningococcal or gonococcal infections or a second episode of bacteremia at any age; high incidence of pneumococcal and meningococcal infections

Major cause of morbidity and mortality after allogenic stem cell transplantation

Graft-Versus-Host Disease (GVHD)

__________ 2-5 weeks post-transplant; erythematous maculopapular rash, persistent anorexia, vomiting and/or diarrhea and abnormal liver enzymes and LFTs;

Acute GVHD:

primary prevention of GVHD is with ________

post-transplant immunosuppressive drugs and corticosteroids

_______develops or persists >3 months after transplant; major cause of non-relapse morbidity and mortality in long-term transplant survivors

Chronic GVHD:

Immune DO Flashcards

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