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Section 5 Immunodeficiency. 1. Primary immunodeficiency. (1) Pure immunoglobulin deficiency ① Bruton-type gammaglobulinaemia ② Hypogammaglobulinaemia of late onset ③ Dysgammaglobulinaemia

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1 primary immunodeficiency
1. Primary immunodeficiency

(1) Pure immunoglobulin deficiency

① Bruton-type gammaglobulinaemia

② Hypogammaglobulinaemia of late onset

③ Dysgammaglobulinaemia

In these disorders these is susceptibility to bacterial and yeast infections, but viral infections are controlled normally. Cell-mediated reactions are intact.

slide3
(2) Pure T-cell deficiency

① Thymic agenesis

② Thymic alymphoplasia (dysplasia)

③ Thymic hypoplasia or aplasia

Here the immunoglobulin levels are normal but there is a complete absence of cell-mediated reactions

(3) Mixed deficiency

2 secondary immunodeficiency
2. Secondary immunodeficiency

Resulting from:

(1) Excessive loss of immunoglobulins

① Protein-losing enteropathy

② Nephrotic syndrome

slide5
(2) Depression of the immune system by

① Old age ② Malnutrition

③Viral infections such as acquired

immunodeficiency syndrome.

④ Leprosy ⑤ Malaria

⑥ Sarcoidosis ⑦ Surgery

⑧ Uraemia

slide6
(3) Immunosuppression by

① X-rays

② Corticosteroids

③ Cytotoxic drugs

④ Antilymphocyte serum

⑤ Anntimetabolits

slide7
(4) Neoplasia

① Hodgkin’s disease

② Multiple myeloma

③ Waldenstrom’s macroglobulinaemia

④ Chronic lymphatic leukaemia

(5) Splenectomy

acquired immunodeficiency syndrome aids
Acquired Immunodeficiency Syndrome (AIDS)

In June 1981, the centers for disease control of the United States reported that five young homosexual men in the Los Angeles area had contracted the AIDS

Etiology: human immunodeficiency virus (HIV)

slide9
HIV

HIV (from Robbins Basic Pathology ,2003)

slide10
Features:

① A long incubation period, followed by a slowly progressive fatal outcome.

② Tropism for hematopoietic and nervous systems

③ An ability to cause immunosuppression

④ Cytopathic effects in vitro.

slide11
Epidemiology

Worldwide: about 10 million people are infected. Five groups of adults at high risk for developing AIDS:

① Homosexual or bisexual males

② Intravenous drug abusers

③ Hemophiliacs

④ Recipients of blood and blood components

⑤ Heterosexual contacts

pathogenesis
Pathogenesis

HIV→CD4+ cell→ CD4+ cell lysis → opportunistic infections and neoplasms

morphology
Morphology

Neither specific nor diagnostic.

① Widespread opportunistic infections

② Kaposi’s sarcoma

③ Lymphoid tumors

slide14
The multiple effects of loss of CD4+ T cells as a result of HIV infection

(From Robbins Basic Pathology ,2003)

Slide 7.41

slide18
AIDS with herpes

Offered by Prof Song W Wong

slide20
HIV infection showing the formation of giant cells in the brain.

(Dr. Dennis Burns) . (From Robbins Basic Pathology ,2003)

Slide 7.40

slide21
Lymph nodes:

Marked follicular hyperplasia (early stages)→ Lymphoid cells depletion (empty-looking lymph nodes or spleen and thymus in later stages).

Mortality: 100%

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