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BAFF-R deficiency

Immunologic phenotype

B cells

Both Baff-R-deficient persons presented with low B cell numbers. The most striking finding is the prominent expansion of transitional B cells (>20% of B cells). Marginal zone like B cells are reduced. Neither patient presented with altered CD21low B cells counts. In both patients no BAFF-R expression could be detected by flow cytometry.

BAFF- Receptor Expression BAFF-R
Click on image to enlarge
Population Marker Altered phenotype * Relative counts ** Absolute counts **
Total B cells   CD19 y - --
Naive B cells   19+ 27- IgD+ IgM+ y - -
Marginal zone like   19+ 27+ IgD+ IgM+ y - --
IgM only memory   19+ 27+ IgD - IgM+ ? ? ?
Switched memory   19+ 27+ IgD- IgM- y - -
IgA+ B cells   19+ IgA+ IgM- ? ? ?
IgG+ B cells   19+ IgG+ IgM- ? ? ?
CD21low B cells   19hi 21low 38low n n n
Transitional B cells   19+ M++ 38++ y ++ +
Plasmablasts   19low M-/+ 38+++ y - -
BAFF-R Show Image CD19+ BAFF-R+ ? ? ?
Pro B cells   Mix neg CD19neg CD22pos ? ? ?
Pre B1 cell   CD22+/CD19+/CyCD79a+/CyIgμ- ? ? ?
Pre B2 cell   CD10+/CyIgμ+/SmIgM- ? ? ?
Immature B cell   SmIgM+/SmIgD- ? ? ?

Legend:

*) Altered phenotype:
Phenotype of respective population is altered compared to adult controls:
y = yes y = no v = variable ? = no information available
**) in all other fields the choices are:
n = normal + = expanded ++ = severely expanded ? = no information available
v = variable - = decreased -- = severely decreased --- = absent
Last update: 2010-05-11 10:53:52

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