[PAGID] diabetes and hypogammaglobulinemia

[Fleisher, Thomas (NIH/CC/DLM) [E]]

DM is not typical of ALPS nor are antibody deficiencies. 

 

Thomas A. Fleisher, M.D.

Chief, Department of Laboratory Medicine

Clinical Center, NIH

Bethesda, MD 20892-1508

Tel 301 496-5668

Fax 301 402-1612

________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Paris, Kenneth
Sent: Friday, May 04, 2007 3:30 PM
To: pagid at list.clinimmsoc.org
Subject: RE: [PAGID] diabetes and hypogammaglobulinemia

 

Hi,

 

Ricardo and I follow a teenage boy with a phenotype consistent with ALPS.  He also has Type I DM since 2-3 years of age.  Our initial workup revealed normal total immunoglobulins, with mild IgG subclass abnormalities and normal response to both protein and polysaccharide antigens.  Interestingly, he then developed recurrent ITP which has been treated with IVIG several times, as well as WinRho.  He is doing well now, with resolution of the thrombocytopenia for the last year or so, and no history of any recurrent infections.   

 

The history below doesn't mention any history of lymphadenopathy, but I might consider ALPS in the differential if there is recurrent lymphadenopathy.  My understanding is the phenotype can be quite varied.  Unlike the patient described below, it is associated with an increase in IgG, but not in all cases from what I've read.  Our ALPS patient had normal total IgG with subclass deficiencies.  There is certainly an association between ALPS and Type I DM and other autoimmune phenomenon (ITP, Evan's Syndrome etc).

 

Ken Paris MD

Dept of Allergy/Immunology

LSU New Orleans

Children's Hospital of New Orleans

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Sorensen, Ricardo
Sent: Fri 5/4/2007 10:55 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] diabetes and hypogammaglobulinemia

Regardless of whatever molecular defect it might be or not be, there is a management question here. We had a girl with hypogammaglobulinemia and type I diabetes whose control was very difficult because of the recurrent infections. She did better better on IgIV (carefully selected not to contain sugars. She was lost to follow-up after Katrina, so I do not have long-term outcome information. 

Ricardo Sorensen

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jack Bleesing
Sent: Friday, May 04, 2007 8:56 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] diabetes and hypogammaglobulinemia

IPEX

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/



>>> 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 

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