IMMUNODEFICIENY DISORDERS Flashcards
Primary immunodeficiencies
B cell disorders, t cell disorders, combined b and t cells, phagocyte disorders, complement disorders
Recurrent infections with pyogenic bacteria indicate a immunodeficiency in
B cells
B cell disorders
X linked/ Bruton’s agammaglobinemia,selective IgA deficiency
T cell disorders
DiGeorge Syndrome, chronic mucocutaneous candidiasis
Combined B and T cell disorders
Severe combined immunodeficiency, wiskott aldrich syndrome, ataxia telangiectasia
Phagocyte disorders
Chronic granulomatous disease, chediak higashi syndrome, leukocyte adhesion deficiency
Complement disorders
Early- C2, C3; terminal- specific deficiency
Very low levels all immunoglobulins; virtual absence of B cells due to tyrosine kinase mutation
Bruton’s aggamoglobinemia
Mgmt for bruton’s
Pooled gamma globulin
Selective IgA deficiency
Due to failure of isotype switching and result to recurrent bacterial sinus and lung infectiob
Defect in B cell maturation to plasma cells
Common variable immunodeficiency
Most common form of severe antibody deficiency affecting both children and adults
Common variable immunodeficiency
Specific T cell deficiency for candida albicans
Chronic mucocutaneous candidiasis
Defect jn IL2 receptors in T cells, ADA deficiency
SCID
Inability to mount IgM response
Wiskott Aldrich syndrome
Mutation in DNA repair enzymes
Ataxia telangiectasia
Lack of NADPH oxidase activity
Chronic granulomatous disease
Chronic granulomatous disease presents with recurrent infections of
Catalase positive bacteria, A. Fumigatis
Failure of phagolysosomal fusion, faulty microtubules impair neutrophil chemotaxis
Chedak higashi syndrome
Defective adhesion (LFA 1) proteins on the surface of phagocytes
Leukocyte adhesion deficiency
Most common complement defect
C2 deficiency
Recurrent infections due to stap aureus
C3 deficiency
Specific deficiency in C5-C9
Terminal complement deficiency
Bacteremia with N. Meningitidis or N. gonorrhea
Terminal complement def
Complement responsible for opsonization
C3B
Complementa that are anaphylatoxin
C3a, C4a, C5a
Complement chemotactic to WBCs
C5a
Complement wc is part of the membrane attack complex
C5b-C9
