[PAGID] diabetes and hypogammaglobulinemia

[Jack Bleesing]

IPEX

JB

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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/



>>> dmvascon at usp.br 5/4/2007 9:30:46 AM >>>

Dear Lawrence

I think the main diagnostic hypothesis is common variable immunodeficiency, but it is necessary to rule out lymphoproliferative disorders and X linked lymphoproliferative disease (SAP deficiency), drugs (such as anticonvulsants, sulphasalazine, gold salts etc), transcobalamin II deficiency and excessive protein loss syndromes. Nowadays his immunologic responsiveness to protein and polysaccharide Ags shows that it is not necessary (now) to replace Immunoglobulins. In case of a CVID(and also other PIDs) there is a risk of development of other autoimmune diseases as well.
I think that could be valuable to try to do the phenotypic and molecular diagnosis of these diseases (SAP, TACI, BAFF, ICOS, TC II) and exclude the use of drugs and malignant disorders such as lymphomas, chronic lymphocytic leukemia and thymoma (these last two are rare in the patients age).

All the best,

Dewton Vasconcelos
University of São Paulo

Jung, Lawrence escreveu: st1\:*{behavior:url(#default#ieooui) }  I  have a 15 y.o. previously healthy Caucasian male patient who was diagnosed to juvenile diabetes last July.  While his diabetes was controlled he started to develop episodes of low grade fever, fatigue and searches for infections were never positive.  IgG was 422   IgA 23 and IgM 26.  [ all low for our lab]IgG1 - 245, IG2 * 30, IgG3 * 22 and IgG4 * 2.   CD3- 1048, CD4 * 585, CD8- 407, CD19 * 534.  He makes normal levels of antibodies to Diphtheria, tetanus and various pneumococcal serotypes.  Neg CMV serology.  EBV VCA IgG + IgM * EBNA +  EA -.Any suggestions as to the etiology of the "low" IgG's and how may it be related to the diabetes?  Is he at risk for developing other autoimmune diseases?   Parents ask whether IVIg should be given to prevent development of the latter.Thanks for your comments.Larry JungOmaha, NE