[CIS-PAGID] panhypogamm in a 22 you

[Routes, John]

agree with SCIG—NK functional studies are not reliable on this amount of immunosuppression---I have a few questions==---what has been the GI workup to date?----what is the stool alpha1-anti-trypsin?---how much prednisone and for how long?=Any assessment of specific Ab production prior to IVIG? =With the lack of infectious complications need to make sure this is not a combination of PLE with steroid induced hypogammaglobulinemia---(Ig levels are c/w agamm but he makes autoAb and has B cells)===Assuming this is CVID with a bad phenotype and considering the autoimmune complications,might be a mutation in TACI---if interested we can sequence for “research” purposes (we are not a CLIA certified lab)-
Jack



John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu




________________________________
From: "Church, Joseph" <JChurch at chla.usc.edu>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Mon, 4 Apr 2011 19:27:50 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo

Hizentra has much lower IgA than Vivaglobin.  Go with the Hizentra.  JC


________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Marianne Empson (ADHB)
Sent: Monday, April 04, 2011 2:49 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo


I have a patient with CVID who started to have systemic reactions to IVIg and was found to have very high anti-IgA antibodies.  Her reactions persisted despite manipulating IV rate, frequency and dose per infusion as well as premed but the features changed from severe chest pain and rigors to severe opiate requiring headaches with every infusion  She has now been on subQ Ig for a year with absolutely no problems.



I would therefore try subQ replacement therapy.



Marianne Empson
Clinical immunologist
Auckland
NZ

________________________________

From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Hare, Nathaniel D [NHare at Cheshire-Med.COM]
Sent: Tuesday, 5 April 2011 7:52 a.m.
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo

He should be tested for X-linked agammaglobulinemia.



Nathaniel D. Hare MD
Allergy & Immunology
CMC - Dartmouth Hitchcock Keene
Keene, NH  03431

ph  (603) 354-5496
fax (603) 354-5498

________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nancy Kingston
Sent: Monday, April 04, 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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