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Table 4 Key characteristics of XLA and CVID

From: X-linked agammaglobulinemia diagnosed late in life: case report and review of the literature

Age of onset usually by 9–18 months usually 2nd – 4th decade
Family Hx of immunodeficiency usually +ve variable*
Inheritance x-linked recessive variable
   Lymph nodes/tonsils absent tonsillar tissue normal tonsillar tissue
   CD19+ B cell numbers markedly decreased/absent normal/low
   CD4+ T cell numbers Normal Variable**
   CD8+ T cell numbers Normal Variable**
   CD4+ CD8+ ratio Variable often decreased
   Specific Antibody titers absent decreased/absent
   Mutations reported Btk TACI, ICOS, BAFF-R, CD19+
Common Complications Infections Infections
  Allergy/Atopy Allergy/Atopy
  CEMA, VAPP -----
  Autoimmunity Autoimmunity
  Malignancy Malignancy
Treatment IGIV IGIV
  Symptomatic care Symptomatic care***
  1. *Some familial clustering has been described in the literature, possibly associated with Class II MHC gene complex
  2. **CD4+ and CD8+ numbers may be low or normal
  3. *** Symptomatic care includes antimicrobials, surgical drainage, nebulizer treatment for wheezing, allergy management, avoidance, nutrition, etc.
  4. Abbreviations: TACI, Transmembrane activator and calcium-modulator and cyclophilin ligand interactor, Btk, Bruton's tyrosine kinase, ICOS, inducible costimulatory receptor, CEMA, chronic enteroviral meningoencephalitis, VAPP, vaccine-associated paralytic poliomyelitis, IGIV, intravenous immunoglobulin.