Disorders of immunity immunodeficiency diseases
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Disorders of Immunity Immunodeficiency Diseases. Professor Sudheer Kher. Classification of Primary Immune Deficiency Syndromes. Disorders of Phagocytosis Chr. Granulomatous Disease Myeloperoxidase Deficiency Chediac- Higashi Syndrome Leucocyte G6PD Deficiency Job’s Syndrome

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Disorders of Immunity Immunodeficiency Diseases

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Disorders of ImmunityImmunodeficiency Diseases

Professor Sudheer Kher


Classification of Primary Immune Deficiency Syndromes

Disorders of

Phagocytosis

Chr. Granulomatous Disease

Myeloperoxidase Deficiency

Chediac- Higashi Syndrome

Leucocyte G6PD Deficiency

Job’s Syndrome

Tuftsin deficiency

Lazy Leucocyte Syndrome

Hyper IgE Syndrome

Actin binding protein deficiency

Shwachman’s disease

  • Disorders of

  • Complement

  • Complement

  • Component deficiencies

  • Complement

    • Inhibitor deficiencies

Disorders of Specific

Immunity

Combined

Immuno

Deficiencies

B & T Cell

Defects

Humoral

Immuno

Deficiencies

B Cell Defects

Cellular

Immuno

Deficiencies

T Cell Defects


Immunodeficiency Diseases

  • Patient unable to fight off infection

  • Hall marks

    • Repeated infections

    • Opportunistic infections


Immunodeficiency Diseases

  • Most are defects in T cells or B cells

    • T cells, Macrophage defects = Fungal, Viral, protozoal infections

    • B cells, Complement defects = Bacterial infections


Immunodeficiency Diseases

  • Congenital

  • Acquired


Congenital

  • B cell deficiency

  • IgA deficiency

  • DiGeorge’s Syndrome

  • Severe Combined Immunodeficiency (SCID)


B Cell deficiency

  • Agammaglobulinemia

  • Hypogammaglobulinemia


IgA deficiency

  • Most common immune deficiency disorder

  • Genetic condition

  • Failure of IgA synthesis

  • Patients have repeated, recurrent sinus, lung, GI infections


DiGeorge’s Syndrome

  • Thymic hypoplasia

  • Severe decrease in T-cell production & function

  • Defects in face, ears, heart.


Severe Combined Immunodeficiency

  • Thymus development arrested at ~ 6-8 weeks gestation.

  • Deficiency, defective maturation of stem cells that produce B & T cells.

  • Little to no antibody production.


SCID

  • Two types

    • Autosomal recessive

    • X-linked disease recessive


SCID

  • Recurrent, frequently overwhelming infections

  • Particularly respiratory and gastrointestinal

  • Most die in first few years of life, usually by one year of age

  • Death usually due to opportunistic infection


Acquired

  • Nutritional

  • Iatrogenic (drugs, radiations)

  • Trauma (prolonged hypoperfusion)

  • Stress

  • Infections (HIV)

  • Malignancies


Immune Deficiency Therapies

  • B-cell deficiency : Gamma globulin

  • SCID : Bone marrow transplant, enzyme replacement

  • DiGeorge’s Syndrome : Fetal thymus transplant

  • Gene therapy


Inherited Functional Disorders

  • Disorders of Granulocyte Function

    • Job’s syndrome – directional motility is impaired – recurrent boils and abscesses

    • Lazy Leukocyte Syndrome – random and directed movement are defective – cells fail to respond to inflammatory stimuli – mild symptoms of low grade fever, recurrent infections (gums, mouth, ears)

    • Chediak-Higashi – abnormal granule release


Inherited Functional Disorders

  • Congenital C3 deficiency – inability to ingest microorganisms- repeated severe infections with encapsulated bacteria

  • Chronic Granulomatous Disease – inability to kill catalase positive organisms – recurrent pyogenic infection

  • Leukocyte Glucose-6-Phosphate Dehydrogenase Deficiency – similar to CGD Myeloperoxidase Deficiency-benign – slow bacterial killing, but complete


B Cell Disorders

  • Infantile sex-linked agammaglobulinemia

    Recurrent bacterial infections

    All classes of immunoglobulin are low

    All lymphocytes are T cells

    Treat with antibiotics and gamma globulin

  • Common Variable hypogammaglobulinemia

    One or a combination of immunoglobulins is missing

    Inability of B cells to mature/function as plasma cells

    Excessive production/activity of T suppressor cells


Monocyte-Macrophage Disorders

  • Mucopolysaccharidoses – deficiencies in specific enzymes to degrade mucopolysaccharides (Hurley syndrome, Hunter syndrome: gargoylism)

  • Lipidoses: lipid storage diseases – macrophages become overloaded with lipids (Gaucher, Niemann-Pick, Tay-Sachs)


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