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Immunodeficiency Disorder, Secondary Immunodeficiency Disorder, Abnormal…

- - - - XLA
      - SELECTIVE lgA Deficiency
      - Immunoglobulin Deficiency with increased lgM
  - - - SCID
        
        Effect both T cells and B cells
        
        defect in bone marrow progenitor cells
        
        defect in signaling molecules or enzymes
        
        genetic defect in ADA
      - WAS
        
        X-linked primary immunodeficiency (T cells & B cells)
        
        genetic defect in X-chromosomes
        
        reduce numbers of platelets (thrombocytopenia) at birth
        
        month later develop eczema
        
        susceptible to bacteria infections
  - - - multi system
      - bacteria, fungi and viruses
      - result from dysfunctional neutropjils
      - present of giant cytoplasmic granules
    - - phagocytes lack the cell surface molecules
      - cannot migrate to the infection site
      - present with severe bacteria infection that spread rapidly
      - mouth & gastrointestinal fluids
    - - defect in NADPH oxidase complex
      - recurrent & uncontrolled bacterial ，fungal infection
      - often fatal
  - - - C1 Inhibitor Deficiency
      - C1,C2 or C4 Deficiency
      - C5-C9 Deficiences
      - Deficiencies in the alternative pathway
      - Decay Accelerating factor
  - - - Result from defective embryogenesis leading to a reduce and defective (or absent) thymus
        Also referred as conginetal thymic hypoplasia
        Individuals with this syndrome have few or no circulating T cells
        
        Clinical Features
        Vulnerable to viral, fungal, and intracellular bacterial infections.
        Experience physical malformations due to developmental issues.
        Malformations: mouth with reverse slant, low-set malformed ears.
        Commonly have cardiac issues and hypocalcemia.
        Peripheral blood lymphocytes show unresponsiveness to T cell activators.
        Improvement often observed with age.
        
        Treatment Transplantation therapy is recommended