Disorders Of The Immune System

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Magor components of immune system are:

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Monocyte خلايا وحيدية

Macrophages خلايا بلعمية

Lymphocytes خلايا لمفاوية

Granulocytic cells خلايا حبيبية(neutrophilsخلايا متعادلة, eosinophilخلايا حمضية, basophilsخلايا قاعدية)

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Mononuclear phagocytes

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Participate in antigen processing, tissue repair, and secretion of mediator fatal to initiation of specific immune responses

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Lymphocytes

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Resonsable for the initial specific recognition of antigen

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Cytotoxic or' killer' T cell

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Responsible for defense against intracellular pathogens

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Neutrophils

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Phagocytose and destroy foreign antigens and microbial organisms

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Eosinophils

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Play a crucial role in the host's defense against parasites .considerably less efficient than neutophils and phagocytosis

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Basophils

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Play an important role in both immediate-and late-phase allergic responses

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Mast cells

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Are basophilic straining cellsاضطراب الخلايا القاعدية:source of many mediator of immediate hypersensitivity

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Thymus function

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To produce T lymphocytes and the site of initial T-lymphocyte differentiation

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Inflammatory mediators

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Histamine,

eosinophil,

neutrophil

, chemoattractans

, proteoglycans and various proteeolytic enzymes

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Histamine

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Bioactive alone; important in the pathogenesis of allergic rhinitis, allergic asthma and anaphylaxis

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Antigens or immunogens

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Foreign substances that can iduce an immune respose

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Immune response

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A complex network of specialized cells, organs, and biologic factors is necessary for the recognition and subsequent elimination of foreign antigens

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Helper T

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The recognition of processed antigen by specialized T lymphoctee

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Activation of B lymphocytes(humoral immune response) and the primary function is

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Synthesis antybodies

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Anybody structure are

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IgG, IgA, IgM, IgD, and IgE

مجوعة في قولك ماجد مع اضافة حرف e

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Hypersensitivity results from

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Inappropriate and sustained production of IgE in response to allergen

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Allergic Rhinitis

التهاب الانف التحسسي

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Characterized by local tissues damage and organ dysfunction in the upper and lower respiratory tract arising from an abnormal hypersensitivity immune response to normally harmless and ubiquitous environment allargens

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Allergic rhinitis phases are

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Early phase: response occurs within minute after exposure to an antigen

Late phase:allergic response my follow the early phase response or may occur as an isolated event

Late phase response is characterized by erthema, in duration, heat burning and itching

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Primary immunodeficiency diseases

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When developmental aberrations in the immune system are genetic in origin and lead to abnormalities in immunocompetence

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Combined Immunodeficiency

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Severe Combined Immunodeficiency Disease presents clinically with an absence of normal thymic tissue, and the lymph nodes, spleen, and other peripheral lymphoid tissues are devoid of lymphocytes.

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SCID

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SCID is a heterogeneous group of disorders characterized by a failure in the cellular maturation of lymphoid stem cells

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Cell mediated immunodeficiency

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Congenital Thymic Aplasia (DiGeorge Syndrome) manifests clinically through defective embryonic development of organs derived from the third and fourth pharyngeal arches, including the thymus, parathyroids, and cardiac outflow tract

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Humoral immunodeficiency

نقص المناعة الخلطية

1- X-Linked Agammaglobulinemia

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Thought to be pathophysiologically and clinically more homogenous than SCID

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Humoral immunodeficiency نقص المناعة الخلطية

2-Common Variable Immunodeficiency Disease

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Often referred to as acquired or adult-onset hypoglobulinemia

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Humoral immunodeficiency نقص المناعة الخلطية

3-Hyper-IgM Immunodeficiency

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Inheritance may be autosomal, although it is most often X-linked

قد يكون الميراث جسمية، على الرغم من أنه في معظم الأحيان مرتبطة X

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Humoral immunodeficiency نقص المناعة الخلطية

Selective IgA Deficiency

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The most common primary immunodeficiency in adults

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Disorders of phagocytic cells

1-Chronic Granulomatous Disease

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Presents with recurrent skin infections, abscesses,خراجات and granulomas; defects in gene coding severely compromise neutrophil killing activity

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Disorders of phagocytic disease

2-Leukocyte Adhesion Deficiency, Type Iنقص التصاق الكريات البيض، النوع الأول

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Autosomal recessive trait; results in impaired trafficking of leukocytes leading to recurrent infections, lack of pus formation, and poor wound healing

سمة المتنحية راثي. يؤدي إلى ضعف الاتجار بالكريات البيض مما يؤدي إلى التهابات متكررة، وعدم وجود تشكيل صديد، وضعف التئام الجروح

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Disorders of phagocyte cell

3-Mendelian Susceptibility to Mycobacterial Disease

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Increased susceptibility to less virulent, nontuberculosis species of mycobacteria; infection with non-typhoidal salmonellae may also be associated

زيادة قابلية الأنواع الفطرية الأقل سلاما من المتفطرة؛ العدوى مع السالمونيلا غير التيفية قد تكون مرتبطة أيضا

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Disorders of phagocyte cell

4-Hyper-IgE Immunodeficiency

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Often referred to as “Job syndrome” since affected individuals suffer from recurrent boils like the biblical figur

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Disorders of phagocyte cell

5-Toll-Like Receptor 3 Deficiency

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Defects in pathway impair viral immunity

العيوب في مسار ضعف المناعة الفيروسية

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Disorders of phagocyte cell

6-AIDS

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Most common immunodeficiency disorder worldwideConsequence of a chronic retroviral infection that produces severe, life-threatening CD4 helper T-lymphocyte dysfunction, opportunistic infections, and malignancy