[PAGID] High CD 19 lymphocytosis and hypogammaglobulinemia

[Richard L. Wasserman, MD,PhD]

We are seeing a five month old girl. Date of Birth Aug 30, 2008, 24 
weeks gestation. Born at an outlying hospital. Neonatologists unwilling 
to transfer. First seen as an outpatient January 5, 2009.

At 3 weeks she was treated for Staph bacteremia.

At 6 weeks she was treated for group B strep sepsis with resolution of 
infection. One week after antibiotics were stopped she developed group B 
strep meningitis; the infection resolved with treatment there have been 
no further infections. There has never been rash, diarrhea, lung disease 
or candidiasis.

Nov 22, 2008
IgA <8, IgG <35, IgM 18

Dec 6, 2008
IgG <35. Neonatologists unwilling to administer IVIG.

Dec 23, 2008
IgA <8, IgG <35, IgM 26
WBC 7.6 ALC 3270, CD 4 28% (abs 1054), CD45RA 23% (abs 864), CD45RO 8% 
(abs 299), CD 8 4% (abs 139), CD19 59% (abs 2083), CD16/56+ 6% (abs 
210),  CD 3 34% (abs 1223), CD 2 35% (abs 1247), HLADR 58% (abs 2217)

Dec 24, IVIG given

January 6, 2009
Unstimulated CD4 7, PHA - CD 4 activation 302
<226 low, 226-524 moderate, >524 high

Con A 768, normal >136
IVIG 1g/kg given

February 3, 2009
WBC 9.6 ALC 5700, CD 4 34% (abs 1944), CD 8 6.6% (abs 376), CD19 60.3% 
(abs 3437), CD 3 38.4% (abs 2189)
IgA 13, IgG 722, IgM 22

Since hospital discharge she has been relatively isolated at home and 
maintained on TMP/SMX prophylaxis. She is growing and gaining weight and 
has had no problems other than being phlebotomized.

Comments, suggestions?
Richard Wasserman
Dallas