I.

Cardinal Signs of Immunodeficiency (10)

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 effect.
4. Two or more pneumonias within 1 year.
5. Failure of an infant to gain weight or grow normally.
6. Recurrent, deep skin or organ abscesses.
7. Persistent thrush 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.

II.

Types of Immunodeficiency (definition)

a. Primary Immunodeficiency diseases
- 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.
b. Secondary Immunodeficiency diseases
- Result from external factors such as infections. More common than
primary immunodeficiencies and frequently occur as a result of
underlying disease processes or from the treatment of these
diseases.

A. Primary Immunodeficiency
1. Phagocytic Dysfunction
- A group of immunodeficiency conditions characterized by
disordered phagocytic activity; they may be extrinsic (e.g.
suppression of the number of phagocytes by immunosuppressive
agents, or dysfunction caused by corticosteroids) orintrinsic (related
to enzyme deficiencies); they are marked by bacterial or fungal
infections that range from mild recurrent skin infection to fatal
systemic infection.
Pathophysiology of Chronic Granulomatous Disease
Chronic Granulomatous
Disease
Severe defect in the
myeloperoxidase-hydrogen
peroxide system

Major means
of bacterial
killing
1

Uses the enzyme

myeloperoxidase,
halides

Phagocytosis

Neutrophils and other

phagocytes switch
much of their glucose
metabolism

It will go to the hexose

monophosphate shunt

A by-product of this
is the conversion of
molecular oxygen

By nicotinamide
adenine
dinucleotide
phosphate (NADPH)

Result in deficient
production of
hydrogen peroxide
and other oxygen
Individuals have adequate
myeloperoxidase and
halide but lack the
necessary hydrogen
peroxide
2

** Individuals with CDG have recurrent severe pneumonias, tumor-like granulomas

in the lungs, skin and bones and other infections.

2. Humoral Disease
a. Brutons Disease
- Plasma cells are lacking, and the germinal centers from all
lymphatic tissues disappear, leading to a complete lack of antibody
production against invading bacteria, viruses, and other pathogens.
Infants born with this disorder suffer from severe infections starting
soon after birth.
- This syndrome is called sex-linked agammaglobulinemia
(Brutons disease) because all antibodies disappear from the
patients plasma.
-

What happens to B cells?

B cells in the peripheral blood and the immunoglobulins IgG, IgM,
IgA, IgD, and IgE are low or absent. The prevalence of this disorder
is approximately 1 case per 100,000 population
What happens to T cells?
normal
What is the missing in this condition that leads to the problem?

Lack of differentiation
of B-cell precursor into
mature B cells

Plasma cells
lacking
Germinal
centers from
all lymphatic

Complete lack
of Antibody
production
against
pathogen

b. Hypogammaglobulinemia
- It is also called common variable immunodeficiency (CVID), a term
that encompasses a variety of defects ranging from
immunoglobulin A (IgA) deficiency, in which only the plasma cells
that produce IgA are lacking.
- Cause: the second type of B cell deficiency results from a lack of
differentiation of B cells into plasma cells
3. Cellular Immunodeficiency
a. DiGeorge syndrome
- Is one example of a primary T-cell immunodeficiency. This rare
congenital disease results from the absence of several genes on
chromosome 22
Pathologic features of thymus:
Cardiac anomaly
Hypocalcemic tetany
Facial abnormalities
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 Renal abnormalities
Facial features:
Cleft palate or lip
Dysmorphic facial features
4. Severe combined immunodeficiency
- Both B and T cells are missing in severe combined
immunodeficiency disease (SCID).
- SCID is a phenotypic term that is used for a wide variety of
congenital and hereditary immunologic defects that are
characterized by early onset of infections, defects in both B- and Tcell systems, lymphoid aplasia, and thymic dysplasia. Inheritance of
this disorder can be X-linked, autosomalrecessive, or sporadic. The
exact incidence of SCID is unknown;
- it is recognized as a rare disease in most population groups, with an
incidence of about 1 case in 1,000,000. This illness occurs in all
racial groups and both genders
a. Wiskott-Aldrich syndrome
- A variation of SCID, is an inherited immunodeficiency caused by a
variety of mutations in the gene coding the WAS protein.
Blood disorders:
Thrombocytopenia with small platelets
Eczema

MANAGEMENT
Bone marrow
transplantation

NURSING
RATIONALE
RESPONSIBILITIES
Monitoring the patients General indicators of
vital signs and blood
circulatory status and
oxygen saturation
adequacy of perfusion.
Providing ongoing
Successful recovery and
support and health
long term wellness
teaching. Include SO
require a coordinated
and family in teaching.
effort by client and
those regularly involved
Place in a private room.
with client.
Limit visitors as
To protect the patient
indicated. Prohibit live
from potential sources of
plants or flowers.
pathogens or infection.
Restrict fresh fruits and
Bone marrow
make sure they are
suppression,
properly washed or
neutropenia, and
peeled. Coordinate
chemotherapy places
patient care so that
the patient at high risk
leukemic patient
for infection.
doesnt come in contact
with staff who also care
4

for patients with

infections or infectious
diseases.
Stem cell

Immunoglobulins

Screen visitors and staff

for signs of infection;
make sure nurse caring
for client with new
transplant is not caring
for another client with
infection. Maintain
protective isolation, as
indicated.
Monitor laboratory tests,
such as WBC count and
blood glucose.
Instruct patients to report
any decrease in urine
output, sudden weight
gain, fluid retention or
edema, or shortness of
breath.
Ask the patient if he can
tolerate the headache or
nausea

Anti-infective/infection
control

Isolation precautions
reduce the possibility of
clients contracting a
nosocomial infection.

Elevated WBC count

signals inflammation of
infection.
Because it is associated
with acute renal failure.

You may have to stop or

slow down the infusion
rate and then restart or
resume it once the
symptoms subside.
Require good hand
Prevents crosswashing protocol for all
contamination and
personnel and visitors.
reduces risk of infection.
Provide thorough skin
Additional measures to
care by keeping the
avoid infection.
patients skin and
perianal area clean,
apply mild lotion or
creams to keep the skin
from drying or cracking.
Thoroughly clean skin
before all invasive skin
procedure.
May indicate local
Inspect skin for tender,
infection. Open wounds
erythematous areas;
may not produce pus
open wounds. Cleanse
because of insufficient
skin with antibacterial
number of granulocytes.
solutions.
Frequent assessment of
Inspect all incisions and
incisions and puncture
5

puncture sites. Evaluate

healing process.

sites promotes early

identification of onset of
infection and prompt
intervention.
Identifying organism
allows for appropriate
Obtain culture specimens
treatment.
of culture drainage, as
appropriate.