IMMUNODISORDERS

1. IMMUNODISORDERS

2. INTRODUCTION
• Immunodeficiency is an abnormal condition of the immune
system in which cellular or humoral immunity is inadequate
and resistance to infection is decreased.
• First evidence of immunodeficiency is an increased
susceptibility to infection.

3. • Immunodeficiency disorders involve an impairment of one
or more of the following immune mechanisms:
• Phagocytosis
• Humoral response
• Cell-mediated response
• Complement
• Combined humoral and cell-mediated deficiency

4. Classification of immune
disorders
Immunodeficiency
disorders
Hypersensitivity
disorders
Autoimmune
disorders

5. FACTORS AFFECTING IMMUNE
SYSTEM
Central nervous
system integrity
General physical
and emotional
status
Medications Dietary patterns
Stress of illness Trauma or
Surgery

6. Immunodeficiency disorders
Immunodeficiency disorders are,
• Primary if the immune cells are improperly developed or
absent, and
• Secondary if the deficiency is caused by illnesses or
treatment

7. Primary immunodeficiency disorders
Primary immunodeficiency disorders are inborn
errors of immune function that predispose people to
frequent, severe infections; autoimmunity; and cancer.

8. Primary immunodeficiency
disorders
• The majority of primary immunodeficiencies are diagnosed in
infancy, with a male-to-female ratio of 5 to 1.
• To date, more than 120 immunodeficiencies of genetic origin
have bee identified.
• Common primary immunodeficiencies include
• Disorders of humoral immunity (affecting b-cell differentiation or
antibody production),
• T-cell defects,
• Combined b- and t-cell defects,
• Phagocytic disorders, and
• Complement deficiencies

9. Phagocytic cells
• Disorder
• Hyperimmunoglobulinemia E (HIE) syndrome
• Major Symptoms
• Bacterial, fungal, and viral infections; deep-seated cold
abscesses
• Treatment
• Antibiotic therapy and treatment for viral and fungal infections
• Granulocyte-macrophage colony stimulating factor (GM-CSF);
granulocyte colony-stimulating factor (G-CSF)

10. B lymphocyte
• Disorder
• Sex-linked
agammaglobulinemia
(Bruton’s disease)
• Major Symptoms
• Severe pyogenic infections
soon after birth
• Treatment
• Passive pooled plasma or
gamma globulin

11. B lymphocyte
• Disorder
• Common variable immunodeficiency (CVID)
• Major Symptoms
• Bacterial infections, infection with Giardia lamblia
• Pernicious anemia
• Chronic respiratory infections
• Treatment
• Intravenous immunoglobulin (IVIG)
• Metronidazole (Flagyl)
• Quinacrine HCl (Atabrine)
• Vitamin B12
• Antimicrobial therapy

12. B lymphocyte
• Disorder
• Immunoglobulin A (IgA) deficiency
• Major symptoms
• Predisposition to recurrent infections,
• Adverse reactions to blood transfusions or immunoglobulin,
• Autoimmune diseases,
• Hypothyroidism
• Treatment
• None

13. B lymphocyte
• Disorder
• IgC2 deficiency
• Major symptoms
• Heightened incidence of infectious diseases
• Treatment
• Pooled immunoglobulin

14. T lymphocyte
• Disorder
• Thymic hypoplasia (DiGeorge syndrome)
• Major symptoms
• Recurrent infections;
• Hypoparathyroidism,
• Hypocalcemia,
• Tetany,
• Convulsions,
• Congenital heart disease,
• Possible renal abnormalities;
• Abnormal facies
• Treatment
• Thymus graft

15. T lymphocyte
• Disorder
• Chronic mucocutaneous candidiasis
• Major symptoms
• Candida albicans infections of mucous membrane, skin, and
nails.
• Endocrine abnormalities (hypoparathyroidism, addison’s
disease)
• Treatment
• Antifungal agents:
• Topical: miconazole
• Oral: clotrimazole, ketoconazole
• IV: amphotericin B

16. Mucocutaneous candidiasis

18. B and T lymphocytes
• Disorder
• Ataxia-telangiectasia
• Major symptoms
• Ataxia with progressive neurologic deterioration, telangiectasia
(vascular lesions), recurrent infections; malignancies
• Treatment
• Antimicrobial therapy;
• management of presenting symptoms;
• fetal thymus transplant,
• IVIG

19. B and T lymphocytes
• Disorder
• Nezelof ’s syndrome (under development of thymus)
• Major symptoms
• Severe infections, malignancies
• Treatment
• Antimicrobial therapy;
• IVIG,
• Bone marrow transplantation;
• Thymus transplantation;
• Thymus factors

20. B and T lymphocytes
• Disorder
• Wiskott-aldrich syndrome
• Major symptoms
• Thrombocytopenia, resulting in bleeding, infections;
malignancies
• Treatment
• Antimicrobial therapy;
• Splenectomy with continuous antibiotic prophylaxis;
• IVIG and
• Bone marrow transplantation

22. B and T lymphocytes
• Disorder
• Severe combined immunodeficiency disease (SCID)
• Major symptoms
• Overwhelming severe fatal infections soon after birth (also
includes opportunistic infections
• Treatment
• Antimicrobial therapy;
• IVIG and
• bone marrow transplantation

23. Complement system
• Disorder
• Angioneurotic edema
• Major symptoms
• Episodes of edema in various parts of the body, including
respiratory tract and bowels
• Treatment
• Pooled plasma, androgen therapy

24. Complement system
• Disorder
• Paroxysmal nocturnal hemoglobinuria (PNH)
• Major symptoms
• Lysis of erythrocytes due to lack of decay-accelerating factor
(DAF) on erythrocytes
• Treatment
• None

25. • Primary immune deficiency causes children and adults to
have infections that come back frequently or are
unusually hard to cure.
• In America alone, up to half a million people suffer from
one or more of the 70 known primary immune deficiency
diseases.

27. SECONDARY
IMMUNODEFICIENCIES

28. Secondary immunodeficiencies
• Secondary immunodeficiencies are more common than
primary immunodeficiencies
• Frequently occur as a result of underlying disease
processes or the treatment of these disorders.

29. Factors
• Intrinsic factors, including
• Immunosuppressive agents,
• Harsh environmental conditions,
• Hereditary disorders other than
primary immunodeficiencies,
and
• Acquired metabolic disorders
that cause secondary
immunodeficiencies

30. Causes Chronic stress
Burns
Uraemia
Diabetes mellitus
Certain autoimmune disorders
Certain viruses
Exposure to immunotoxic medications and chemicals
Self-administration of recreational drugs and alcohol.

31. Secondary Immunodeficiency
disorders

32. Medical Management
• Diagnosis and treatment of the underlying disease
process.
• Treatment of the primary condition often results in the
improvement of the affected immune components
• Factors that contribute to immunosuppression are
identified and infection is treated.
• Additional treatment includes HSCT, monoclonal
antibody therapy, and anticoagulation therapy as
indicated.

33. NURSING
MANAGEMENT
Assessment
Patient
teaching
Selected
interventions
Supportive
care

34. Assessment
• History
• Infection
• Cause
• Factors
• Examinationn
• Signs and
symptoms
Intervention
• Vitals
• Lab
investigations
• Reduce the risk
of infections
• Nutrition
• Maintain general
functions
• Bowel
• Respiratory etc.
• Psychological
support
Teaching /Home
care
• Disease
condition
• Hygiene
• Treatment
• Follow up