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. Author manuscript; available in PMC: 2016 May 17.
Published in final edited form as: J Allergy Clin Immunol Pract. 2015 Nov 7;4(1):38–59. doi: 10.1016/j.jaip.2015.07.025

TABLE III.

Classification schemes defining subgroups of patients with CVID on the basis of flow cytometric B-cell immunophenotyping*

Nearly absent B cells (<1%†) Includes all patients with
  severe defects in B-cell
  differentiation
Low switched memory B cells (<2%) Indicates a defective germinal
  center development
  similar to
CD27+IgM−IgD−   • ICOS deficiency
  • CD40L deficiency
Increased risk:
  • Splenomegaly
  • Granulomatous disease
Expansion of transitional B cells (>9%)
  CD38hiIgMhi 		Associated with
  lymphadenopathy
Expansion of CD21low B cells (>10%) Associated with splenomegaly

ICOS, Inducible T cell co-stimulator.

*

Adapted from Wehr et al.29

†

Expressed as a percentage of total B cells (CD19+ or CD20+).