Section 5 Immunodeficiency

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Section 5 Immunodeficiency
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1. Primary immunodeficiency

• (1) Pure immunoglobulin deficiency
• ? Bruton-type gammaglobulinaemia
• ? Hypogammaglobulinaemia of late onset
• ? Dysgammaglobulinaemia
• In these disorders these is susceptibility to
  bacterial and yeast infections, but viral
  infections are controlled normally. Cell-mediated
  reactions are intact.

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• (2) Pure T-cell deficiency
• ? Thymic agenesis
• ? Thymic alymphoplasia (dysplasia)
• ? Thymic hypoplasia or aplasia
• Here the immunoglobulin levels are normal but
  there is a complete absence of cell-mediated
  reactions
• (3) Mixed deficiency

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2. Secondary immunodeficiency

• Resulting from
• (1) Excessive loss of immunoglobulins
• ? Protein-losing enteropathy
• ? Nephrotic syndrome

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• (2) Depression of the immune system by
• ? Old age ? Malnutrition
• ?Viral infections such as acquired
• immunodeficiency syndrome.
• ? Leprosy ? Malaria
• ? Sarcoidosis ? Surgery
• ? Uraemia

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• (3) Immunosuppression by
• ? X-rays
• ? Corticosteroids
• ? Cytotoxic drugs
• ? Antilymphocyte serum
• ? Anntimetabolits

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• (4) Neoplasia
• ? Hodgkins disease
• ? Multiple myeloma
• ? Waldenstroms macroglobulinaemia
• ? Chronic lymphatic leukaemia
• (5) Splenectomy

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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)

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HIV
HIV (from Robbins Basic Pathology ,2003)
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Features

• ? A long incubation period, followed by a
  slowly progressive fatal outcome.
• ? Tropism for hematopoietic and nervous
  systems
• ? An ability to cause immunosuppression
• ? Cytopathic effects in vitro.

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• Epidemiology
• Worldwide about 10 million people are
  infected. Five groups of adults at high risk for
  developing AIDS
• ? Homosexual or bisexual males
• ? Intravenous drug abusers
• ? Hemophiliacs
• ? Recipients of blood and blood components
• ? Heterosexual contacts

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Pathogenesis

• HIV?CD4 cell? CD4 cell lysis ? opportunistic
  infections and neoplasms

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Morphology

• Neither specific nor diagnostic.
• ? Widespread opportunistic infections
• ? Kaposis sarcoma
• ? Lymphoid tumors

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The multiple effects of loss of CD4 T cells as a
result of HIV infection
(From Robbins Basic Pathology ,2003)
Slide 7.41
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AIDS with Kaposi sarcoma
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Kaposi sarcoma in liver of AIDS patients
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AIDS with herpes Offered by Prof Song W Wong
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AIDS-related Marked follicular hyperplasia (early
stages)
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HIV infection showing the formation of giant
cells in the brain. (Dr. Dennis Burns) . (From
Robbins Basic Pathology ,2003)
Slide 7.40
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• 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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