Immunodeficiency

Rashmita Devi
Tutor
AIIMS Kalyani
 Introduction
• Immunodeficiency disorders may be caused by
a defect or deficiency in phagocytic cells, B
lymphocytes, T lymphocytes, or the
complement system
Classification
 Primary immunodeficiency
• Primary immunodeficiencies, 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
• These disorders may involve one or more
components of the immune system
 PHAGOCYTIC DYSFUNCTION
• Primary defects of phagocytes are genetic in
origin and affect the innate immune system
• In some types of phagocytic disorders, the
neutrophils are impaired so that they cannot exit
the circulation and travel to sites of infection.
• In some disorders, the neutrophil count may be
very low; in others, it may be very high because
the neutrophils remain in the vascular system.
 Clinical Manifestations
• Increased incidence of bacterial and fungal
infections
• ( fungal :Candida viral: herpes simplex or
herpes zoster virus.)
• One type of phagocytic disorder, hyper
immuno globulinemia E (HIE) syndrome,
formerly known as Job syndrome
• Severe neutropenia may be accompanied by
deep and painful mouth ulcers, gingivitis,
stomatitis, and cellulitis
 Job syndrome
• Job Syndrome (Hyper-IgE
syndrome) is a rare, primary
immunodeficiency
distinguished by the clinical
triad of atopic dermatitis,
recurrent skin staphylococcal
infections, and recurrent
pulmonary infections.
• The disease is characterized by
elevated IgE levels with an early
onset in primary childhood
 Assessment and Diagnostic Findings
• History
• Lab test :
1. Nitroblue tetrazolium reductase test
a blood test that measures the ability of the
immune system to convert the colorless
nitroblue tetrazolium (NBT) to a deep blue.
 Medical Management
• Treating bacterial infections with prophylactic
antibiotic therapy
• treatment for fungal and viral infections
• Granulocyte transfusion
• Granulocyte-macrophage colony-stimulating
factor (GM-CSF) or granulocyte colony-
stimulating factor (G-CSF)
• (these proteins draw non lymphoid stem cells
from the bone marrow and hasten their
maturation.)
 B-CELL DEFICIENCIES
• Two types of inherited B-cell deficiencies.
• The first type 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.
• The second type of B-cell deficiency results from
a lack of differentiation of B cells into plasma
cells.
• Only diminished antibody production occurs
with this disorder.
 Clinical Manifestations
• Recurrent pyogenic infections usually occur by 5
to 6 months of age
• More than half of patients develop pernicious
anemia.
• Lymphoid hyperplasia of the small intestine and
spleen and gastric atrophy detected by biopsy of
the stomach are common findings.
• Other autoimmune diseases, such as arthritis and
hypothyroidism, frequently develop in patients.
 Diagnostic evaluation
• Sex-linked agammaglobulinemia may be
diagnosed by the marked deficiency or
complete absence of all serum
immunoglobulins.
• Antibody titers to confirm successful
childhood vaccination are determined by
specific serologic tests.
 Medical Management
• Intravenous immunoglobulin (IVIG)
• Antimicrobial therapy is prescribed for respiratory
infections to prevent complications such as pneumonia,
sinusitis, and otitis media.
• Intestinal infestation with G. lamblia is treated with a
10-day course of metronidazole (Flagyl) or a 7-day
course of quinacrine hydrochloride (Atabrine)

• Pernicious anemia receive parenteral injections of

vitamin B12 at monthly intervals.
• Management may also include physical therapy with
postural drainage for patients with chronic lung disease
 T-CELL DEFICIENCIES
• Defects in T cells lead to opportunistic infections.
• Most primary T-cell immunodeficiencies are
genetic in origin.
• An increased susceptibility to infection is
common.
• Symptoms can vary considerably depending on
the type of T-cell defect.
• Because the T cells play a regulatory role in
immune system function, the loss of T-cell
function is usually accompanied by some loss of
B-cell activity
 Continued..
• Di George syndrome, or thymic hypoplasia, is
one example of a primary T-cell
immunodeficiency.
• Chronic mucocutaneous candidiasis with or
without endocrinopathy is another T-cell
disorder associated with a selective defect in
T-cell immunity; it is thought to be caused by
an autosomal recessive inheritance
 Clinical manifestations
• Infants born with DiGeorge syndrome have
hypoparathyroidism with resultant
hypocalcemia resistant to standard therapy
• congenital heart disease, characteristic facial
features, and possibly renal abnormalities.
• The initial presentation of chronic
mucocutaneous candidiasis may be either
chronic candidal infection or idiopathic
endocrinopathy
• Patients may survive to the second or third
decade of life.
• Problems may include hypocalcemia and
tetany secondary to hypofunction of the
parathyroid glands
 Assessment and Diagnostic Findings
• The status of T cells can be evaluated by
peripheral blood lymphocyte counts
• Because T cells constitute 65% to 85% of
peripheral blood lymphocytes,
• Dermal sensitization of the patient or
stimulation of the patient’s T cells in vitro may
be conducted to determine if the T cells are
capable of producing the expected responses.
 Management
• Patients with T-cell deficiency should receive
Pneumocystis. carinii prophylaxis
• General care includes:
• management of hypocalcemia
• Correction of cardiac abnormalities.
• Hypocalcemia is controlled by oral calcium
supplementation in conjunction with vitamin
D or parathyroid hormone administration
• Transplantation of the fetal thymus, postnatal
thymus, and human leukocyte antigen (HLA)-
matched bone marrow has been used for
permanent reconstitution of T-cell immunity.
• IVIG therapy may be used if an antibody
deficiency exists. This therapy may also be
used to control recurrent infections
Combined B-cell and T-cell deficiencies

• These are those disorders of the immune

system that have elements of dysfunction of
both the B cells and T cells.
• A variety of inherited (autosomal recessive
and X-linked) conditions fit this description.
• These conditions have in common disruption
of the normal communication system of B
cells and T cells and impairment of the
immune response
• These conditions generally appear early in life.
• Ataxia-telangiectasia is an autosomal
recessive disorder affecting both T- and B-cell
immunity.
• In 40% of patients with this disease, a
selective IgA deficiency exists.
• IgA and IgG subclass deficiencies, along with
IgE deficiencies, have been identified.
• Both B and T cells are missing in severe
combined immunodeficiency disease (SCID).
• Inheritance of this disorder can be X-linked,
autosomal recessive, or sporadic
• Wiskott-Aldrich syndrome is a variation of
SCID compounded by thrombocytopenia (loss
of platelets).
 Clinical Manifestations
• The onset of ataxia (uncoordinated muscle
movement)
• telangiectasia (vascular lesions caused by
dilated blood vessels) usually occurs in the
first 4 years of life
 Continued..
• The onset of symptoms :first 3 months of life
• respiratory infections, pneumonia (often
secondary to P. carinii), thrush, diarrhea, and
failure to thrive.
• Maculopapular and erythematous skin rashes
may occur.
• Vomiting, fever, and a persistent diaper rash
are also common manifestations
 Medical Management
Treatment of ataxia-telangiectasia :
• Early management of infections with
antimicrobial therapy
• Management of chronic lung disease with
postural drainage and physical therapy, and
management of other presenting symptoms.
 Continued..
• Other treatments :
Transplantation of fetal
thymus tissue, gland
• IVIG administration
• Stem cell and bone
marrow transplantation
 DEFICIENCIES OF THE
COMPLEMENT SYSTEM
• Alterations in normal components of
complement can result in increased
susceptibility to infectious diseases and to
immune mediated disorders.
• Angioneurotic edema: caused by an inherited
deficiency of the inhibitor of C1 esterase,
which opposes the release of inflammatory
mediators.
• It results in frequent episodes of urticaria and
edema in various parts of the body
 Secondary Immunodeficiencies
• Secondary immunodeficiencies are more common than
primary Immunodeficiencies
• Frequently occur as a result of underlying disease processes
or from the treatment of these diseases.
Common causes
• Malnutrition,
• Chronic stress, burns, uremia, diabetes mellitus
• Autoimmune disorders, certain viruses, exposure to immuno
toxic medications and chemicals, and self-administration of
recreational drugs and alcohol
• AIDS, the most common secondary immunodeficiency disorder
• Patients with secondary immunodeficiencies have
immunosuppression and are often referred to as
immunocompromised hosts.
 Management
• Diagnosis and treatment of the underlying
disease process.
• Other:
• Eliminating the contributing factors
• Treating the underlying condition
• Using sound principles of infection control.
 Nursing Management for Patients
With Immuno deficiencies
• Assessment of the patient for infection and for
response to treatment is important if it is to
be effective.
• Nursing care of patients with primary and
secondary immuno deficiencies depends on
the underlying cause of the
immunodeficiency, the type of
immunodeficiency, and its severity.
 Assessment
• The assessment focuses on

History of past infections,

particularly the type and
frequency of infection
Signs and symptoms of any
current skin, respiratory,
gastrointestinal, or
genitourinary infection; and
measures that prevent
infection
 Continued..
• The nurse monitors the patient for
:
 fever; chills; cough with or without sputum;
shortness of breath;
 difficulty breathing; difficulty swallowing; white
patches in the oral cavity;
 swollen lymph nodes;
 nausea; vomiting; persistent diarrhea;
 frequency, urgency, or pain on urination;
 redness, swelling, or drainage from skin wounds;
 lesions on the face, lips, or perianal area;
persistent vaginal discharge with or without
perianal itching; and persistent abdominal pain
 Continued..

Vital signs
Development of pain, neurologic signs,
cough, and skin lesions are monitored and
reported.
 Pulse rate and respiratory rate should be
counted for a full minute, as even subtle
changes can signal deterioration in the
patient’s clinical status.
Assessment also focuses on nutritional
status; stress level and coping skills; use
of alcohol, drugs, or tobacco; and
general hygiene, all of which may affect
immune function.
Strategies the patient has used to
reduce risk for infection are identified.
 Other aspects of nursing care
• Directed toward reducing the patient’s risk for
infection
• Measures aimed at improving immune status
and treating infection, improving the nutritional
status, and maintaining bowel and bladder
function.
• These include careful hand hygiene,
encouraging the patient to cough and perform
deep-breathing exercises at regular intervals,
• protecting the integrity of the skin and mucous
membranes.
• Health care personnel must use strict aseptic
technique when performing invasive
procedures, such as dressing changes,
venipunctures, and bladder catheterizations.
• Other aspects of nursing care include assisting
the patient in managing stress and in adopting
a lifestyle that enhances immune system
function