Immunodefeciency pptx - د. علياء

Immunodeficiency

Dr. Alya Al Zobair
Radiation and Medical Oncologist

Immune deficiency

Immunodeficiency: is a state in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent.

Immunocompromised patient :A person who has an immunodeficiency of any kind, may be particularly vulnerable to opportunistic infections.

An opportunistic infection is an infection caused by pathogens (bacteria, viruses, fungi, or protozoa) that take advantage of an opportunity not normally available, such as a host with a weakened immune system.

Immune system

Immune deficiency

Classification:
Specific
Non specific
1-Primary (rare): genetically determined deficiencies, mostly present in childhood or adolescence
2-Secondary (common)
Consequences:
Recurrent infections, autoimmunity and susceptibility to malignancy

Primary immune deficiency
A- Primary phagocyte deficiencies:

B-Complement pathway deficiencies:

C-Primary deficiencies of the adaptive immune system:
i- Primary T-lymphocyte deficiencies:
ii- Combined B- and T-lymphocyte immune deficiencies:
iii- Primary antibody deficiencies:

Secondary immune deficiencies

Secondary immune deficiencies are much more common than primary immune deficiencies.
CAUSES:
Physiological; Pregnancy, aging , prematurity.
Infection; HIV, Measles, Mycobacterium infection.
Iatrogenic; Immunosuppressive therapy, Antineoplastic agents, Corticosteroids, Stem cell transplantation, Radiation injury, Some anti-epileptic agents.
Malignancy; B-cell malignancies including leukaemia, lymphoma and myeloma, thymoma.
Biochemical and nutritional disorders; Malnutrition, Renal insufficiency/dialysis, Diabetes mellitus, Specific mineral deficiencies, e.g. iron, zinc.
Other conditions; Burn, Hyposplenism.

Presenting problems in immune deficiency

Frequent, severe infections or infections caused by unusual organisms or at unusual sites.

*Oral Candidiasis
*Mouth Ulceration
*Herpes simplex

Presenting problems in immune deficiency

Warning signs of primary immune deficiency: The presence of ≥ 2 warning signs may indicate an underlying primary immunodeficiency:
• ≥ 4 new ear infections within 1 yr
• ≥ 2 serious sinus infections within 1 yr
• ≥ 2 mths on antibiotics with little effect
• ≥ 2 pneumonias within 1 yr
• Failure of an infant to gain weight or grow normally
• Recurrent, deep skin or organ abscesses
• Persistent thrush in mouth or fungal infection on skin
• Need for intravenous antibiotics to clear infections
• ≥ 2 deep-seated infections, including septicaemia
• A family history of primary immune deficiency

Immune deficiencies and common patterns of infection

Baseline investigations when immune deficiency is suspected:
1-Full blood count with white cell differential,
2-Acute phase reactants (CRP),
3-Renal and liver function tests,
4-Serum immunoglobulins .
5-Additional microbiological, viral load ( HIV) and radiological tests may be appropriate.

HIV infection (Aids)
The acquired immunodeficiency syndrome (AIDS) is caused by the human immunodeficiency virus (HIV), which progressively impairs cellular immunity.
In 2011 it was estimated that there were 34.2 million people living with HIV/AIDS, 2.5 million new infections and 1.7 million deaths
Transmission of HIV:
1-sexual contact, Worldwide, heterosexual is the major route of transmission.
2- Exposure to blood (e.g. injection drug use, occupational exposure in health-care workers)
3-Blood products,
4-Mother to child..
.

hiv

HIV is an enveloped ribonucleic acid (RNA) retrovirus from the lentivirus family. After mucosal exposure, HIV is transported to the lymph nodes via dendritic cells, where infection becomes established. This is followed by viraemia and dissemination to lymphoid organ.
HIV can only infect and destroy cells bearing the CD4 receptor; these are T-helper lymphocytes, which are pivotal in orchestrating the immune response, the patient becomes susceptible to opportunistic diseases. The predominant opportunist infections in HIV-infected people are the consequences of impaired cell-mediated rather than antibody-mediated immunity (e.g. mycobacteria, herpes viruses)..
The host immune response to HIV infection is both humoral, with the development of antibodies to a wide range of antigens, and cellular, with a dramatic expansion of HIV-specific CD8 cytotoxic T lymphocytes, resulting in a CD8 lymphocytosis

Clinical features of hiv infection

HIV itself is associated with a wide variety of clinical
manifestations, in addition to features of opportunistic diseases .
Fever, weight loss, lymphadenopathy, mucocutanous manifestation, Kaposi’s sarcoma, Anaemia, arithritis.
GIS symptoms: diarrhea and esophageal candidiasis.
Neurological symptoms: meningitis, neuropathy and cognitive impairment.

ORAL MANIFESTATION OF HIV INFECTION
1- Fungal lesion :
a- candidiasis
b- histoplasmosis
2- Viral lesion:
a- herpes simplex
b- herpes zoster
c- CMV
e- hairy leukoplakia (EPV)
3- Bacterial lesion:
periodendal disease
4- Neoplastic lesions:
Kaposi’s sarcoma
lymphoma.
5- Oral ulceration: ophthous ulcer.