ALTERATION IN IMMUNOLOGIC RESPONSE

OVERVIEW OF THE IMMUNE SYSTEM

ANATOMY AND PHYSOLOGY
mmune System- the body protects itself from foreign invaders, such as bacteria, viruses, parasites,
and fungi, through the organs and cells of the immune system.
- Components work together to recognize "selffrom "non-selfand to rid the body of those
substances recognized as "non-self.
Functions:
1. Defense against physical injury and infection.
2. Maintenance of homeostasis.
mmune System Organs: described as lymphoid because they're all involved with the growth,
development, dissemination of lymphocytes.
1. Bone marrow
2. Lymph nodes
3. Thymus
4. Spleen
5. Tonsils, adenoids and other mucoid lymphatic tissues
6. Hematopoietic system
Bone marrow
A specialized soft tissue filling the spaces in cancellous bone of the epiphyses. t is responsible
for:
4 Releasing mature B lymphocytes into the blood circulation
4 Moving T lymphocytes from bone marrow to the thymus.
Lymph Nodes
Small, oval-shaped structures located along a network of lymph channels.
Help remove and destroy antigen that circulate in the blood and lymph.
Filter lymphatic fluid and return it to the bloodstream.
Filter foreign invaders such as viruses and bacteria and can serve as waiting stations for
lymphocytes that might be needed in that area to fight infection.
Thymus
uses hormones to enable maturation of lymphocytes, produced by bone marrow, into T
lymphocytes (Tcells), the mature Tcells can then function normally.


SpIeen
functions as reservoir for blood and blood cells.
Also like a screen to filter out unwanted invaders and help break-up old RBC's that lose their
elasticity with age and can't squeeze through the final mesh of the spleen.
Good for filtering out one specific bacteria the $treptococcus pneumonia.
TonsiIs
Together with adenoids and other mucoid lymphatic tissues defend the body against
microorganisms.
In the hematopoietic system, bone marrow and lymphatic tissues produce blood cells, including those
involved in immunologic defense.
Nonspecific ImmunoIogic Defense
s a type of immunity effective against any harmful agent entering the body.
The body's natural immunity can discriminate friend from foe or "selfor "nonselfbut cannot
distinguish between agents and pathogens.
NaturaI Mechanisms incIude:
1. Physical barriers
ntact skin and mucous membranes prevent pathogens from gaining access to the
body.
Cilia of the respiratory tract filter and clear pathogens from the upper respiratory
tract.
2. Chemical barriers
Acidic gastric juices, enzymes in tears, and saliva, sebaceous and sweat secretions
attempt to destry invading bacteria and fungi.
3. Biologic response modifiers
nterefron, a viricidal substance, counters viruses and activates other components of
the immune system
4. Actions of white blood cells
Neutrophils- are the first to arrive at the inflammatory injury
Eosinophils and basophils- are activated in response to allegic reactions and stress.
Granulocytes- release cell mediators, such as histamine, bradykinin and
prostaglandins and engulf the foreign toxins.
Monocytes or macrophages- functions as phagocytic cells to engulf, ingest and
destroy foreign toxins.
5. nflammatory response
This mechanism is elicited in response to tissue injury or invading organisms.
Mast cells release chemical mediators, which enhance the inflammatory response
and produce the typical signs of infection.
Vasoconstriction and vasodilation also play a role in the inflammatory response.
6. Natural killer (NK) cells.
These lymphocytes are responsible for immune surveillance and host resistance to
infections

. Complement System
Composed of several proteins that are important in the inflammatory process
t's activated by the antigen-antibody complexes (classic pathway or toxins released
by antigens (alternate pathway).
One of the body's primary defenses; it immediately assists in mast cell degradation,
which enhances vascular permeability and assist in attracting neutrophils to the site.
Functions:
4 Cell lysis
4 Opsonization
4 Chemotaxis
4 Agglutination
4 Neutralization of viruses
Activation of complement can occur in one of two basic ways:
4 CIassicaI- antigen-antibody complex activate C1 (1
st
of circulating
complement proteins
4 AIternate- no antigen-antibody complex is required; complement can be
initiated by the release of endotoxins and begins with C3.
Specific ImmunoIogic Defense
A type of immunity effective against specific harmful agents entering the body.
mmunity is a normal adaptive response designed to protect the body against potentially harmful
antigens.
Types of Immunity
1. nborn mmunity- is an inherited immunity of species, races and individuals to certain diseases.
2. Acquired mmunity- is immunity that develops as an individual encounter specific harmful agents. t
may be natural or artificial and active or passive.

1. Active Immunity- involves the production of antibodies and sensitized lymphocytes in response
to an antigen or immunization.
a. Memory ceIIs- do not secrete antibodies, but on reexposure to the specific antigen, they
develop into antibody-secreting plasma cells.
b. Important vaccines- include an intramuscular tetanus and diphtheria booster injection,
which is required for adults at least 10 years, and the hepatitis B vaccine, which is
required for all health care workers.
2. Passive immunity-is a temporary immunity acquired by introduction of antibodies or sensitized
lymphocytes from another source. The bodies does not generate memory cells.
The mechanisms of Specific Immunity
Two Types: (discriminate self from non self and distinguish among antigens)
1. Humoral mmunity- involves in the formation of antibodies by plasma cells in response to foreign
proteins.
- functions primarily in Type , and hypersensitivity reactions

Immune System CeIIs
Produced in bone marrow.
The main cells are:
1. B cells (B lymphocytes)
2. Phagocytes
3. T cells (T lymphocytes)
B cells
involved in humoral immunity, produced antibodies.
n humoral, a B cell will divide and differentiate into plasma cells when it encounters its
triggering antigen.
Unsensitized B cells
Proliferate and mature into plasma cells after exposure to antigen
Plasma cells
Differentiate into memory cells and antibodies.
mmunoglobulin- some antibodies are protein that perform special functions.
Five types:
1. gA- defends external body surfaces and is present as secretory antibodies in colostrums, saliva,
tears, and nasal fluids as well as respiratory, G, genitourinary secretions.
-protects the mucous membranes and internal cavities against infection.

2. gD- found on the surface of B cells and functions in controlling lymphocytes activation or
suppressions
-Fetal antigen receptor.

3. gE- antibody responsible for hypersensitivity reactions; it has an immediate response to an antigen
and stimulate the release of heparin and histamine from mast cells.
- Causes allergies, drug sensitivity, anaphylaxis, and immediate hypersensitivity.
- Combats parasitic diseases.

4. gG-makes up the majority of plasma antibodies and is the main antibacterial and antiviral antibody.
- Transfusion of gG specific for viral diseases, such as varicella, are useful in treating children
who are exposed but have decreased immune functions.
- Causes placental barrier
- Causes certain immunological diseases.
- Found in blood and lymph

5. gM- the first immunoglobulin produced during an immune response; because it's very large.
- Usually seen only in the blood and can't pass into tissues to fight infections.
- Dominant antibody in ABO incompatibility.


Note: All antibodies are immunoglobulin but not all immunoglobulin are antibody.
2. Cell-mediated immunity- involves attack of microbes by special killer T cells formed from
lymphocytes.
- functions primarily in delayed hypersensitivity reactions; rejection of
transplants; and viral, fungal and chronic infections.
T ceIIs
Responsible for cell-mediated immunity.
Lymphocytes derived from bone marrow migrate to thymus, where they mature into T cells.
n cell-mediated immunity, T cells directly attack antigens, including bacteria, viruses, and other
pathogens.
Cell-mediated immunity is also responsible for tissue transplant incompatibility rejections and
delayed hypersensitivity reactions (positive tuberculin test response)
Different types of T cells work together to create the best immune response possible:
1. Helper Tcells (CD4+cells) stimulate B cells to mature into plasma cells that produce
immunoglobulins to fight antigens and to also remember them if they occur again in the future.
2. The helper Tcells also help the killer T cells (CD8 + cells) to more readily recognize the antigen and
attack directly.
3. Killer Tcells bind to the surface of the invading antigen and disrupt the cell membrane, causing the
antigen's destruction.
Other types:
1. Suppressor T cells suppress B cell synthesis of antibody production through a feedback
mechanism
2. Memory T cells store future immune response to some antigen
3. Cytotoxic T cells directly attack antigen, altering cell membrane with resultant lysis.
Stages of Specific Immune Responses
1. Recognition- circulating lymphocytes and macrophages recognize foreign material or antigen as non
self.
2. Proliferation- sensitized lymphocytes proliferate, differentiate and mature into respective T and B cells.
3. Response- antibody is produced with specific T-cell action.
4. Effector- antigen is destroyed by antibody, which is produced by B-cells or cytotoxic T cell action.

ASSESSMENT
< HeaIth History
< Elicit a description of the present illness and chief complaint, including onset, course, duration,
location and precipitating and alleviating factors.
< Elicit a description of the client's overall health status, including immunization status, usual
childhood diseases, known allergies and a history of past and present medications.Cardinal
signs and symptoms indicating altered immunity are subsequently described.
General
a. Recurrent infections

b. Seasonal symptoms
c. Weight loss
d. Fever
Head
a. tching, burning, watering eyes, vision problems, and eye infections
b. Recurrent ear infections
c. Rhinitis and Sneezing
Respiratory system
a. Cough
b. Dyspnea
c. Recurrent infection
Cardivascular system
a. Pain
b. Raynaud phenomenon
Gastrointestinal system
a. Nausea and vomiting
b. Diarrhea
Genitourinary system
a. Recurrent infections
b. Dysuria and hematuria
Musculoskeletal system
a. Weakness and fatigue
b. nability to perform activities of daily living
Neurologic sytem
a. Disorientation to name, date and place
b. Altered level of consciousness
c. Paresthesias
< Explore the client's health history for risk factors associated with immune disorder including not keeping
up to immunizations, exposure to infectious disease and exposure to pollens, insects and allergens.

2. PhysicaI Examination
a. nspection
1. nspect the skin and mucous membrane for rashes, lesions, dermatitis, purpura and any type
of inflammation or drainage.
2. Assess the joints for tenderness, edema and range of motion.
3. nspect ear for drainage, inflammation, and scarring from ear infections.
b. Palpation
1. Palpate the anterior node and posterior cervical, axillary and inguinal lymph node for
enlargement.
2. Note the location, size, and consistency of lymph node. Document complaints of tenderness if
the node is palpable.

c. Auscultation
1. Auscultate lungs for abnormal lung sounds such as wheezing, crackles and ronchi.
2. The heart sounds for abnormalities such as palpitations, and dysrhythmias.
3. Laboratory and Diagnostic Findings
a. Multi-allergen allergy testing
Measures the quantity of allergen specific gE antibodies and is done to identify allergens to
which the client has immediate hypersensitivity
b. T- and B-lymphocytes assays
Evaluate the number of lymphocytes in the immune system
c. mmunoglobulin Assays (gG, gA, and gM)
Can detect and monitor immune deficiencies.
d. Serum complement assays test
Test for C3 and C4 complement when the total complement level is decreased.
e. Autoantibody tests
Antinuclear antibody (ANA) test-measures and differentiates ANA associated with certain
autoimmune diseases such as SLE.
Rheumatoid factor (RF) test measures for a macroglobulin type of antibody found in rheumatoid
arthritis.
f. Radioallergosorbent test (RAST)
A radioimmunoassay that measures allergen specific gE.
g. The human immunodeficiency virus (HV) test
Determines the presence of antibody to HV, which is the etiologic factor for ADS.
. Nursing Diagnoses
1. neffective Airway Clearance
Assess respiratory status including the lung, rate and depth of respirations, effort of breathing,
use of accessory muscles, cyanosis, restlessness, anxiety or any change in level of
consciousness.
2. Risk for nfection
nstruct the client on ways to avoid infection, including the importance of personal hygiene and
avoidance of people with infections and large crowds.
nstruct to wash affected area with warm water before applying topical creams, wash hands
before and administering topical creams.

. Medications used to treat Immune ProbIems
1. Antihistamines
< Help relieve symptoms of allergic rhinitis and allergic dermatoses by inhibiting histamine
release.
< Example:
Diphenhydramine HCL (Benadryl)
Chlorpheniramine Maleate (Teldrin)
< Nursing mplications:
Advise client not to engage in activities that require mental alertness.
Should not take medication with alcohol.

2. Bronchodilator: Sympathomimetics
< Used to treat Asthma and Allergic rhinitis, exert effect on beta 2 receptors by relaxing smooth
bronchial muscle and dilating airways.
< Example:
Albuterol sulfate (Ventolin)
Terbutaline sulfate (Bricanyl)
Epinephrine (Bronkaid, Epipen)
sopproterenol (suprel)
< Nursing mplications:
Clients at risk for anaphylaxis should wear medical alert bracelet.
Clients at risk for anaphylaxis should carry emergency epinephrine.

3. Bronchodilator: Xanthine Derivative
< Used to relax bronchial smooth muscle
< Example:
Aminophylline (Amoline)
Theophylline (Theo-dur)
Ephedrine (Efedron)

4. Cromolyn Sodium (ntal)
< Administered as prophylactic therapy for asthma

5. mmunosuppressive Agents
< Lessen or prevent an immune response
< Example:
Azathioprine (muran)
Cyclosporine (Sandimmune)
< Nursing mplications:
Avoid used in pregnancy
Take pc to decrease G upset
Should not be given to clients with active infection
Avoid exposure to infection, wash hands frequently.
Avoid immunization
nstitute protective isolation/ reverse isolation.




6. Corticosteroid
< Used to treat severe immune or inflammatory reactions.
< Example:
Hydrocortisone (Solu-cortef)
Dexamethasone (Decadron)
Prednisone (Deltasone)
Methylprednisolone (Solumedrol)

. mmunomodulating Agents
< Bind to the cells infected with the HV
< Example:
Zidovudine (Retrovir)
Didanosine (Videx)
Zalcitabine (Hivid)

8. Antiinflammatory Agents
< Administered to reduce inflammatory and pain
< Example:
Acetylsalicylic Acid (aspirin)
buprofen (Advil)
ndomethacin (ndocin)

9. Common Managements for Patients with mmunologic Disorders
< -eep the patient away from sources of infection/allergen when necessary
NO fresh flowers!
Limit visitors
Animals, dusts, sprays, and perfumes
Remember:
O Effective HANDWASHNG is the single most important measure in infection
control.
< U-se hot-cold applications to inflamed sites
< L-oad the medications as prescribed
< A-rrange for dietary consult to ensure optimal nutrition
< N-ote vital functions
Vital signs
ECG
ABG values
Urine output
< I-ntervene immediately to relieve respiratory distress as needed
Maintain an open airway
Administer oxygen as indicated
Position the client to maximize lung expansion
Prepare to assist with emergency tracheostomy if necessary.



CATEGORIES OF IMMUNE DISORDERS
A. Hypersensitivity Reactions- are immune responses to allergens that result in tissue destruction
1. Type 1 (Anaphylactic) reactions- are mediated by immunoglobulin E antibody, which
promotes the release of histamine and other reactive mediators. These basophil or mast
cells produce the characteristics symptoms of asthma or hay fever.
2. Type (Cytotoxic) reactions- like in hemolytic anemia, are mediated by gG and gM
antibodies, which attach to cells (usually circulating blood elements) and cause cell lysis.
3. Type (immune complex) reactions- like rheumatoid arthritis and serum sickness, are
mediated by antigen-antibody complexes that deposit in the lining of blood or on tissue
surfaces.
4. Type V (delayed hypersensitivity) reactions- like in contact dermatitis, transplant rejections)
are mediated by lymphokines released sensitized T lymphocytes

B. Allergic Disorders- are hypersensitive responses to an allergen to which an organism has previously
been exposed and to which the organism has developed antibodies
1. nteractions between antigens and antibody typically results in one or more manifestations
of tissue injury
2. gE antibodies are fromed by persons experiencing allergies who are genetically
predisposed. Histamine and other mediators are released on re-exposure to the allergen to
which the person is sensitized.

C. Autoimmune Disorders- are conditions in which the body no longer differentiates self from non-self.
1. Alteration in T cells or B cellsproduce autoantibodies and autosensitized T cells that cause
tissue injury. These changes may involve one organ or many organ systems.
2. The cause of autoimmune disorders remain unknown, but many theories exist.

D. mmune Deficiency-is defined as a congenital or acquired deficit in the immune system that makes the
person susceptible to life threatening ooportunistic infection
1. Congenital (primary) immunodeficiency- the body produces inadequate amounts of one or
more immune cells. Deficits can be humoral (B cell), cell mediated (T cell), or combined.
2. Acquired (secondary) immunodeficiency- is attributed to various etiologies including
a. mmunosuppressive therapy, such as chemotherapeutic agents, corticosteroid, non
steroidal anti-inflammatory agents, and irradiation.
b. Age related factors, such as deterioration in the thymus gland and T cell functioning
and a decreased number of suppressor T cells and helper T cells.
c. Disruption of skin integrity, as occurs with burns and trauma.
d. Nutritional deficits
e. Malignant processes, such as leukemia and lymphoma
f. nfectious processes, such as sepsis and ADS.





DISORDERS OF THE IMMUNE SYSTEM
DEFINITION OF TERMS
AggamagIobuIin
Disorder marked by an almost complete lack of immunoglobulins or antibodies.
AIIergen
Substance that causes manifestations of allergy.
AIIergy
nappropriate and often harmful immune system response to substances that are normally harmless.
Anergy
Loss or weakening of the body's immunity to an irritating agent or antigen.
Antibody
Protein substances developed by the body in response to and interacting with a specific antigen.
Antigen
Substances that induces the production of antibodies.
ChemicaI Mediator
Biochemical secretion of specialized cell that facilitate localization of infection.
< Bradykinin: polypeptide that stimulates nerve fibers and causes pains.
< Histamine: substances in the body that causes increased gastric secretion, dilation of capillaries and
constriction of the bronchial smooth muscle.
< Leukotrienes: a group of chemical mediators that initiate the inflammatory response.
< Serotonin: chemical mediator that acts as a potent vasoconstrictor and bronchoconstrictor
< Interferons: proteins formed when the cells as a potent vasoconstrictor and bronchoconstrictor
activating other components of the immune system.
< ProstagIandin: stimulates inflammatory response.
< CompIement:A type of plasma protein, which is made in the liver and activated when an antibody
couples with its antigen.
< HypogammagIobuIinemia: lack of one or more of the five immunoglobulins; caused by B-cell
deficiency
< Immunocompetent: a client whose immune system is able to identify antigens and effectively destroy
or remove them.
< PanhypogIobuIinemia: general lack of immunoglobulins in the blood.

1. IMMUNODEFICIENCY
mmunodeficiency disorders may be caused by a defect or deficiency in phagocytic cells, B
lymphocytes, T lymphocytes, or the complement system.
The cardinal symptoms of immunodeficiency
, Poor response to treatment of infections
, nfections caused by unusual organisms or organisms that are normal body flora.
, Chronic or recurrent severe infections
, Chronic diarrhea
Types:
Primary Immunodeficiencies
< Are genetic in origin and are caused by intrinsic defects in the cells of the immune system
< Rare disorders with genetic origins
< Are seen primarily in infants and young children
< Symptoms usually develop early in life after protection from maternal antibodies decreases

1. Phagocytic Dysfunctions
The neutrophils are impaired are impaired so that they cannot exit the circulation and
travel to site of infection.
As an result the patient cannot mount a normal inflammatory response against
pathogenic organism.
n some disorder, the neutrophils count may be very low; in others, it may be very high
because the neutrophils remains in the vascular system.
S/Sx:
4 Sometimes Asymptomatic
4 ncreased incident of bacterial and fungal infections because of severe
neutropenia.
Recurrent furunculosis
Cutaneous abscess
Chronic eczema
Bronchitis
Pneumonia
Chronic otitis media
Sinutis
Death from overwhelming infection
4 Hyperimmunoglobulinemia E (HE) syndrome, formerly known as Job
Syndrom, white blood cells cannot produce an inflammatory response to the
skin infections; this results in deep-seat cold abscesses that lack the classic
signs and syntoms of inflammation (redness, heat, and pain).
4 Diagnosis:
Diagnosis is based on the history: Failure of an infction to resolve with
visual treatment is also an important indicator.
Nitroblue Tetrazolium Reductase Test (NTRT): Laboratory analysis of
the cytocidal (causing the death of cells) activity of the phagocytic
cells.
4 MedicaI Management:
Longterm Antibiotic therapy and treatment for viral and fungal infection;
Granulocyte transfusion, although used, are seldom successful of the
short half-life of the cells; Treatment with granulocyte-macrophage
colony-stimulating factor (GM-CSF) or granulocyte colony-stimulating
factor (G-CSF).

mmune Serum Globulin(about 100-400 mg/kg IV every 3-4 wks)


Proqnoi:
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1. Eight or more new ear infections within 1 year.
2. Two or more serious sinus infections within 1 year.
3. Two or more Months on antibiotics with little effects.
4. Two Or more pneumonias within 1 year.
5. Failure of an infant to gain weight or grow normally.
6. Recurrent, deep skin or organs abscesses.
. Persistent thrust in mouth or elsewhere on skin, after age 1.
8. Need for intravenous antibiotics to clear infections.
9. Two or more deep-seated infections such as meningitis, osteomyelitis, cellulitis, or sepsis.
10. A family history of primary immune deficiency.

/c .-oo, .,o c /-.-; /oo/--o; /- A/o/
1. Two or more new ear infections within 1 year.
2. Two or more serious sinus infections within 1 year, absence of allergy.
3. One pneumonia per year for more than 1 year.
4. Chronic diarrhea with weight loss.
5. Recurrent viral infection (cold, herpes, warts, condyloma).
6. Recurrent need for intravenous antibiotics to clear infection.
. Recurrent, deep abscesses of the skin or internal organs.
8. Persistent thrush or fungal infection on skin on skin or elsewhere.
9. nfection with normally harmless tuberculosis-like bacteria.
10. A family history of P.

2. B-ceII Deficiencies
a. Bruton's Disease
, a:sex linked Aggamaglobulinemia
, Results from lack of differentiation of B-cell precursors into mature B cells.
, As a result, plasma cells are lacking, leading to a complete lack of antibody
production against invading bacteria, viruses, and other pathogens.
, nfant with sex-linked aggamaglobulinemia usually become symptomatic after the
natural loss of maternally transmitted immunoglobulins, which occurs after at
about 5 to 6 months of age.
, B cells in the peripheral blood and the mmunoglobulin M,A,G,E and D are low or
absent.
, nfants born with this disorder suffer from severe infections starting soon after
birth.
, S/Sx:
4 Severe infections soon after birth
4 Recurrent pyogenic infections usually occur by 5 to 6 months of age.
, Medical Management
4 Passive pooled plasma or gammaglobulin

-. CommonVaria-e Immunodeficiency (CVID)
, a: Hypogammaglobulinemia
, Results from a lack of differentiation of B cells into plasma cells.
, Only diminished antibody production occurs with this disorder.
, S/Sx
4 Hypoplasia of the peyers pathches and spleen may be detected through
biopsy
4 Frequent infections caused by:
< Haemophillus influenza
< Streptococcus pneumonia
< Staphylococcus aureus
4 Pernicious Anemia
, Management
< V immunoglobulin
< Metronidazole (Flagyl)
< Quinacrine HCl (Atabrine)
<
Vitamin B
12
< Antimicrobial Therapy
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c. Immunogo-uin (Ig Deficiency
, Plasmacells that produce gA are lacking
, S/Sx:
4 Predisposition to recurrent infections
4 Adverse reactions to blood transfusions or immunoglobulin.
4 Autoimmune diseases
4 Hypothyroidism
, Management:
4 NONE
4 Supportive Management
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d. IgC2
, S/Sx: Heightened incidence of infectious diseases
, Management: Pooled immunoglobulin

3. T-ceII deficiencies
Most are genetic in origin
The loss of T-cell function is usually accompanied by some loss of B-cell activity.
An increased susceptibility to infection is common

a. DiGeorge Syndrome
, a: Thymic Hypoplasia
, This are congenital disease results from the absence of several
genes on chromosome 22
, T cell deficiency occurs when the thymus gland fails to develop
normally during embryogenesis.
, S/Sx: CATCH 22
4 C-ardiac abnormality (specially Tetralogy of Fallot)
4 A-bnormal facies
4 T-hymic aplasia
4 C-left palate
4 H-ypocalcemia (resistant to therapy) due to
hypoparathyroidism
, Dx:
4 Peripheral Blood Lymphocyte CountLymphopenia
, Management:
4 Pneumocystis carinii prophylaxis
4 Hypocalcemia
Lifelong Oral calcium supplementation
Lifelong Vitamin D
Parathyroid hormone administration
4 Correction of cardiac cardiac abnormalities
Dx---Tx
4 Transplantation of matched thymus and/or bone marrow
uman Leukocyte Antigen (LA)-genetic markers
found on chromosome 6 that are used for tissue
"typing.
4 Vg


-. Chronic Mucocutaneous Candidiasis
, Prolonged candida albicans infections of mucous membrane, skin
and nails.
, Management
4 Antifungal agents:
Topical:miconazole
Oral:clotrimazole, ketoconazole
V:amphotericin B


. Com-ined B and T ce ymphocytes Deficiencies
Disorders of the immune system that have elements of dysfunction of both
the B cells and T cells


a. taxia-Teangiectasia
, An autosomal recessive disorder affecting both T-and B-cell
immunity
, S/Sx:
4 Progressive cerebellar ataxia (uncoordinated muscle
movement)
4 Telangiectasia (vascular lesions caused by dilated blood
vessels)
4 Recurrent bacterial infection of the sinuses and lungs
, Management
4 Ensure safety of the client
4 Antimicrobial therapy
4 Postural drainagepneumonia
4 Thymus transplantation
4 Vg

-. Nezeof's Syndrome
, Characterized by absent T cell function, deficient B cell function
and fairly normal g levels.
, S/Sx:
4 Severe, recurrent and eventually fatal infections
(pneumonia, otitis media, chronic fungal infection, URT,
diarrhea and hepatosplenomegaly)
4 Malignancies
4 Weight loss and poor eating habits
, Management
4 Antimicrobial therapy
4 Vg
4 Bone Marrow Transplantation
4 Thymus transplantation
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c. Wisott-drich Syndrome
, S/Sx:
4 Thrombocytopenia, resulting in bleeding
4 nfections
4 Malignancies


, Management:
4 Antimicrobial therapy
4 Splenectomy with continuous antibiotic prophylaxis;
4 Vg
4 Bone Marrow Transplantation
This is an X-linked recessive condition therefore this is almost exclusively in boys

d. Severe Com-ined Immunodeficiency Disease (SCID)
, Both B and T cells are missing
, Cause is unknown
, S/Sx
4 Respiratory infections, pneumonia (often secondary to P.
carinii)
4 Thrush
4 Maculopapular and erythematous skin rashes
4 Vomiting, fever, and a persistent diaper rash
4 Diarrhea, and
4 Failure to thrive
, Management:
4 Antimicrobial therapy
4 Vg
4 Bone Marrow Transplantation

. CompIement System Disorder
Compement
< Is a group of at least 20 (C1, C2, C3...C20) circulating plasma
proteins, made in the liver, that are sequentially activated in the
presence of antigen.
< unctions:
psonization
Cell lysis
Chemotaxis
Agglutination
eutralization of invaders
Anaphylatoxinstimulation of inflammatory response
< Activation of Complement $ystem
Classical Activation
O (+)AgAb complex (C1 and C2 activated)
Alternate Activation
O (+)Ag but (-) AgAb complex (C3-C20 are activated)

a. ngioneuritic Edema
, s caused by an inherited deficiency of the inhibitor of C1
esterase, which opposes the release of inflammatory mediators,
thereby edema
, t may result from food or drug allergy, infection, or emotional
stress or it may be hereditary.

, S/Sx:
4 Frequent urticaria in various parts of the body
4 Episodes of edema in various parts of the body, including
respiratory tract, face, neck, lips, larynx hands, feet,
genitalia and bowels.
4 Management:
Pooled plasma