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-R

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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		19^hi 21^low 38^low	n	n	n
Transitional B cells		19⁺ M⁺⁺ 38⁺⁺	y	++	+
Plasmablasts		19^low M^-/+ 38⁺⁺⁺	y	-	-
BAFF-R	Show Image	CD19⁺ BAFF-R⁺	?	?	?

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

Immune phenotyping in primary immunodeficiency

BAFF-R deficiency

Immunologic phenotype

B cells

Legend:

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