Internal Medicine

0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40

Immunodeficiency Disorders 819

■Gingivitis, skin infections, organ abscesses, lymphadenitis: neu-

trophil/phagocyte defect

■Viruses (herpes, varicella, CMV): T-cell deficiency

■Fungi

➣Candida: T-cell deficiency

➣Aspergillus: T-cell/phagocyte defects

■Parasites

➣Giardia lamblia: B-cell deficiency

➣Pneumocystis carinii: T-cell deficiency

■Bacteria

➣Encapsulated organisms: B-cell or complement deficiency

➣Low-virulence organisms: neutrophil/phagocyte defects

■Diarrhea, malabsorption: T- or B-cell deficiency

Signs & Symptoms

■Eczema: Wiskott-Aldrich syndrome, hyper-IgE syndrome

■Abscesses: neutrophil/phagocyte defects

■Telangiectasia: ataxia-telangiectasia

■Partial oculocutaneous albinism: Chediak-Higashi syndrome

■Candidiasis: T-cell deficiency

■Digital clubbing, rales: B-cell, T-cell deficiency

■Diffuse lymphadenopathy: neutrophil/phagocyte defects

■Lupus-like syndrome: complement deficiencies

■Ataxia: ataxia-telangiectasia

tests

Lab Tests

■Evaluation of B-cell immunity

➣Quantitative serum immunoglobulins: IgG, IgM, IgA

➣IgG subclasses are usually not helpful; test results vary among

different labs

■Specific antibody titers

➣Isohemagglutinins (IgM antibodies toA&Bblood groups)

➣Antibodies to protein antigens (tetanus, diphtheria): IgG1 sub-

class

➣Antibodies to polysaccharide antigens (Pneumococcus,

Haemophilus influenzae): IgG2 subclass

■B-cell enumeration

➣CD19 surface marker

■Evaluation of T-cell immunity

➣T-cell enumeration