[PAGID] CVID/HIGM/ALPS

[Berger, Melvin]

Does he have any IgE ?  
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Lisa Kobrynski, MD, MPH
Sent: Thu 8/7/2008 10:45 AM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] CVID/HIGM/ALPS



I was hoping to get some suggestions on a patient of mine
I am following a 10 year old boy who presented initially with lymphadenitis and
bronchiectasis at age 4 years.  He had malakoplakia found by the pathologists in
his tonsillar tissue after a tonsillectomy.

He initially had low IgG and IgA with a normal IgM and very poor specific
antibody responses and was given a diagnosis of CVID.
Over the years he has had assorted infections (yersinia, salmonella enteritis,
pneumonia) but developed very significant cervical lymphadenopathy last year.
Repeated biopsies have not shown any malignant transformation or clonality of
the cells.  One node became necrotic and grew achromobacter xylosidans, which he
has had intermittently in the blood ever since.  It is not resistant to
everything.  Also his IgM is now over 3000.  ALPS panel initially showed an
increase in DN T cells, activated T cells and a decrease in memory B cells.  DN
T cell % had decreased at the last measurement.
He has minimal hilar adenopathy, some mild inguinal adenopathy, but the cervical
LN are the biggest issue.

I want to take a poll and find out who thinks this is just CVID with
lymphoproliferation?  SHould we treat him with chemotherapeutic agents to
suppress the lymphoproliferation?
Could this have been ALPS all along?
WHo thinks this is a HyperIgM - AID or UNG defect?  (We checked CD40 and CD40L
and they were normal)

Thanks
Lisa

Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Division of Allergy and Immunology
Emory University
404-727-3575
404-727-5045 (fax)




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