[CIS-PAGID] panhypogamm in a 22 yo

[Nancy Kingston]

Thanks every one for your comments so far.  
I had initially been reserved about using Hizentra because in the drug insert it warns against using in patients with low IgA / IgA antibodies.  Yes, I would much rather pursue Subcutaneous IgG replacement as would the patient, so I think we can move forward with a trial of this.
The patient has been off and on steroids and I have checked the Ig's off steroids, still all low.
GI diagnosed the patient with neutropenic enterocolitis.  He has undergone ileocecal resection in the past.  He also has a h/o a tongue mass with a bx report c/w hyperplastic tonsillar tissue.  He has a negative w/u for celiac in the past as well.
In 2003 he had a high RF>640 (<20 IU/ml).  There is a negative ANA.  Felty syndrome had been mentioned in a previous report, but looks like not pursued.
I think that the point about Rituxin is a good suggestion.  I don't think this had been pursued yet because he has been doing fairly well in the last few months on current therapy.
So, the plan now is to move forward with Hizentra, investigate the GI issues more thoroughly as well as the rheumatologic issues.  I do think TACI mutation might be an option, so thanks for that offer.  I was also considering HIGM.  I would like to look at more B cell markers as well.

--- On Tue, 4/5/11, Berger, Melvin <Melvin.Berger at UHhospitals.org> wrote:


From: Berger, Melvin <Melvin.Berger at UHhospitals.org>
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo
To: pagid at list.clinimmsoc.org
Cc: "Kerns, Leigh A" <Leigh.Kerns at UHhospitals.org>
Date: Tuesday, April 5, 2011, 1:24 PM


You could try Sub-cu IgG with Hizentra, which has very low IgA. 

Has he had endoscopy ?   We have had a couple of patients with severe lymphonodular hyperplasia in the intestines, which had a lot of plasmacytoid elements. They were losing a lot of protein and lymphocytes into the gut, and one in particular, was referred because of difficulty keeping up his serum IgG despite relatively high IV doses. He had already had splenectomy for hemolytic anemia, and problems wityh steroids. When he was given Rituxin for a flare of the hemolytic anemia, his IgG markedly went up, and his dose could be reduced. Presumeably, the rituxin killed the B-lineage cells in his gut, and his leaking got much better. 

Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Nancy Kingston
Sent: Mon 4/4/2011 3:37 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo


Dear Colleagues,
An interesting patient was recently referred to me from an oncology colleague who had been seeing him for autoimmune neutropenia and thrombocytopenia.  He had also treated him for panhypogammaglobulinemia with Gammagard.  He was having anaphylaxis to the product and so replacement IgG was stopped.He had been previously dx'd with CVID.

The patient's chief complaint is not infections at all.  He comes with a history mainly of autoimmune cytopenias, on Mepron, Cellcept, and prednisone.  He also has diarrhea fatigue and a 60 pound weight loss in the last couple of years.  CBC/diff is currently ok. No endocrine issues or rashes. The patient and his family had previously been advised to not get primary immunizations.

No family hx of immune defect or consanguinity

Has had mild splenomegaly, no hepatomegaly.
He has had his tonsils and appendix removed.

Recent labs:
IgG 74-77 (578-1228 mg/dl)
IgA <6 (59-337 mg/dl)
IgM <4-9 (23-166 mg/dl)
IgE <4 (0-150 mg/dl)
Anti-IgA Ab IgG-377.6 (<52 U neg)
CD3, CD4, CD8, CD19 total numbers are WNL
CD16 abs # 23 (24-911)
Decreased NK cell function by the Cincinnati lab
No antibody titers for H. influenza, tetanus, 23 serotypes of S. pneumonia, or EBV.
Total complement is normal.
Hx of Negative ALPS testing.

I would appreciate any further opinions you might share regarding gammaglobulin replacement and/or testing for this patient.
Thank you for your input.
Nancy Wasserbauer, DO
Akron Children's Hospital Dept of Allergy and Immunology




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