[PAGID] WAS and CD8

[Jack Bleesing]

Ditto in our post-BMT population. Lisa is reviewing our results in WAS-BMT.

JB

---------------------------------------------------------------------------
Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> "Luigi Notarangelo" <luigi.notarangelo at childrens.harvard.edu> 1/10/2008 1:38:30 PM >>>

Jack:

Also all of my WAS have the same phenotype. Not only that, but patients with
WAS who attain a mixed chimerism after SCT tend to have optimal
reconstitution with donor-derived T cells in the CD8 compartment. So, it
looks that WASP plays a role in CD8 T cells development and/or survival.

Luigi Notarangelo


Luigi D. Notarangelo, M.D.
Director, Research and Molecular Diagnosis Program on Primary
Immunodeficiencies
Division of Immunology, Children's Hospital
Professor of Pediatrics and Pathology, Harvard Medical School
Karp Building, 9th floor, Rm 09210
1 Blackfan Circle
Boston, MA 02115
USA

(tel) (617)-919-2276
(fax) (617)-730-0709


Secretary: Luisa Raleza
email: luisa.raleza at childrens.harvard.edu 




On 1/10/08 12:46 PM, "Jack Bleesing" <Jack.Bleesing at cchmc.org> wrote:


> Ashish:

> 

> What is a normal number of FOXP3+ CD4+ T-cells for an 8 month old?

> 

> Funny thing: all our WAS patients have a high CD4:CD8 ratio (>5), typically

> due to lack of CD8+ T-cells.

> 

> 

> 

> ---------------------------------------------------------------------------

> Jack J.H. Bleesing, M.D., Ph.D.

> Cincinnati Children's Hospital Medical Center

> Division of Hematology/Oncology

> 3333 Burnet Avenue, MLC 7015

> Cincinnati, OH 45229

> 513-636-4266 (phone)

> 513-636-3549 (fax)

> Jack.Bleesing at CCHMC.org 

> http://www.cincinnatichildrens.org/immunodeficiencies/ 

> 

> 

>>>> <kumar036 at umn.edu> 1/10/2008 11:51:57 AM >>>

> Dear Friends,

> 

> I am looking for help with a 8 month boy who has severe eczema, failure to

> thrive and significant eosinophilia. His total white count is 25-30 with

> 25% eos, giving him an absolute eosinophil count of 7000. He has widespread

> eczema which on biopsy showed infiltration with eos and lymphocytes. Total

> B, T and NK numbers are in the normal range with a CD4 to 8 ratio of 6. IgG

> of 200, and IgE of 70. IgA and IgM are normal (31). He has had watery

> diarrhea since birth, weighs only 5 Kg (at 8 months), no diabetes or

> thyroid dysfunction. Given this picture, I suspected IPEX even though he

> has no endocrinopathy, but his FOXP3+ CD4s are normal in number.

> 

> Where do I go next with this picture? There is no positive family history.

> I am waiting maternal engraftmetn studies, in case this is SCID or Omenn

> with maternal engraftment. I also just found that his CH50 is 0, with

> normal C3 and C4 levels. I am going to repeat the CH50 and get C2 and C5

> levels. There are few papers from the 70s describing Leiner's disease with

> C5 deficiency that causes eczema and failure to thrive. His hair looks

> normal and he doesn't have any teeth yet. He does not look dysmorphic and

> hasn't had any infections besides a couple URIs.

> 

> Ashish Kumar

> University of Minnesota

> 

> 

> 

>