[PAGID] High CD 19 lymphocytosis and hypogammaglobulinemia

[Dewton Vasconcelos]

This is a very interesting case.

About the hypogammaglobulinemia I would think about prematurity, as 
placental transfer of IgG occurs mainly in the last three months of 
gestation. Certainly hypogamma could explain the bacterial infections.
The most interesting feature in my point of view is the 
immunophenotyping with high B cells, near normal NK and low T cells with 
very low CD8 cell counts. With that phenotype I would think initially in 
ZAP70, MHC I and CD8alpha deficiency, but we could not exclude 
IL-7Ralpha and PNP deficiencies in a leaky form - CD45 already 
excluded). Lymphoproliferative assays with normal results are suggestive 
of normal (or near normal T cells). It is interesting that HLA-DR 
expression is almost equal to B cells; do you have HLA-DR in T CD4 
cells, as a marker of activation?
Thinking in a clinical point of view I would follow-up the patient very 
nearly and looking for complications and evolution of immunophenotyping, 
as we have seen some patients with CIDs that eventually improved 
spontaneously.

Best regards,

Dewton

Dewton de Moraes Vasconcelos, MD, PhD
University of São Paulo Medical School


Richard L. Wasserman, MD,PhD escreveu:

> 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

>

>

>

>


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