[CIS PIDD] [cis-pidd] Vici Syndrome

[CIS-PIDD]

Thanks for the reply. I can't find recurrent splenic abscesses clearly associated but my understanding is that the immune issues related to the syndrome are broad. The abscesses have come and gone over the past year and he has been treated with long term broad spectrum abx. Recent aspiration was sterile. He also has intestinal failure and would have to receive abx prophy IV instead of PO PCN.  I can certainly bring up the idea of a partial splenectomy. Thanks again for your thoughts.



On Dec 7, 2016, at 8:29 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hi, Allison.

Are splenic abscesses associated with Vici syndrome?  And, do you know the cause of the abscesses?

It would be a shame to add asplenia to the kid’s immune issues.

Any chance of a partial splenectomy?

Joe Church
Children’s Hospital Los Angeles

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, December 07, 2016 1:27 PM
To: CIS-PIDD
Subject: [cis-pidd] Vici Syndrome

Dear Colleagues,

We are caring for a 2 year old male with Vici syndrome. The most pressing issue is recurrent splenic abscesses.  A splenectomy has been recommended and we have been asked to comment.

He has lymphopenia, normal IgG and IgM, elevated IgA (586), low CH50 (25), decreased TLR3 function, normal neutrophil oxidative burst assay, 1/14 pneumococcal titers >1.3, good diphtheria response, tetanus was low, decreased percentage of transitional B cells and absent isotyped switched memory B cells.

We have ordered a lymphocyte proliferation assay, repeat CH50, immunoglobulins and an SPEP. He has recently received TDap, Hib, flu, meningococcal and prevnar vaccines.

We reviewed: https://www.ncbi.nlm.nih.gov/pubmed/21965116

Does anyone have experience treating immunodeficiency in Vici syndrome, specifically related to splenectomy?

Thanks,
Allyson

Allyson Larkin, MD
Assistant Professor of Pediatrics
Training Program Director, Allergy & Immunology Fellowship
Division of Pulmonary Medicine, Allergy & Immunology
4401 Penn Avenue
AOB, Suite 3300
Pittsburgh, PA 15224

Phone: 412-692-7885
Fax: 412-692-8499

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