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IMMUNODEFICIENCY DISORDER - Coggle Diagram
IMMUNODEFICIENCY DISORDER
PRIMARY IMMUNODEFICIENCY DISORDER
Combined T cell and B cell disorder (15-20%)
Severe combined immunodeficiency disorders (SCID)
Genetic defect
Enzyme adenosine deaminase (ADA)
Defect in signaling molecules
Defect in bone marrow progenitor cells
Wiskott Aldrich Syndrome (WAS)
Genetic defect in X-chromosomes
Thrombocytopenia at birth
Phagocytic disorders (20%)
Chediak Higashi Syndrome
Dysfunctional neutrophils
Multi system immunodificiency
Present of giant cytoplasmic granules
Chronic granulomatous Disease (GCD)
Often fatal
Inherited-Defect in NADPH oxidase complex
Leukocyte Adhesion Deficiency ( LAD)
Cannot migrate to site of infection
Phagocytes lack the cell surface
T cells disorder (10%)
DiGeorge's syndrome (congenital thymic hypoplasia)
Genetic defect
Chromosome 22
Clinical features
Physical malformations
Cardiac abnormalities & hypocalcemia
Peripheral blood lymphocytes do not respond to the polyclonal T cell activators
Defective embryogenesis leading to a reduce and defective thymus
Complement system disorder (2-4%) O
C5-C9 Deficiency
Deficiencies in alternative pathway
C1, C2 or C4 Deficiency
Decay Accelerating Factor
C1 Inhibitor Deficiency
B cell disorder (50%)
Immunoglobulin Deficiency with increased IgM
Decrease in IgG, IgA, IgE
Defect in T cell helper
Genetic defect
CD40L
Clinical features :
Several bacterial infections
Fungal-like infections
Selective IgA Deficiency
Low level of serum IgA
B cell do not differentiate to IgA secreting plasma cells
Clinical features
Allergies
GI Tract disease
X-linked Agammaglobulinemia (XLA) also known as Bruton's agammaglobulinemia
Genetic defect
Single gene that code throsine kinase
PreB do not develop to mature B cells
Clinical features
Infant-recurrent upper respiratory tract infections
Viral infection
INTRODUCTION
Causes
Decrease in cell number and excess production of molecules
Secondary Immunodeficiency Disorder
Primary Immunodeficiency Disorder
SECONDARY IMMUNODEFICIENCY DISORDER
Acquired immunodeficiency disorder
Viral infections
AIDS
Therapeutic drugs
Tumor / malignancies treated by TOXIC DRUGS ---> NOT cancer specific ---> normal cells are DESTROYED ---> cause nonspecific immunosuppression
Malnutrition and disease
Affect both cellular & humoral immunity
Deficiencies in specific vitamins
Normal physiological changes
Occur in pregnancies and aging
Depression on immune response
Abnormal production of immunological effectors
Caused by tumor / malignancies
CONCLUSION
Treatment
Managing symptoms and preventing infections
Medication
Immunoglobulin therapy
Lifestyle changes
Early diagnosis and management
improve quality of life
Preventing complications
