[CIS PIDD] [cis-pidd] Vici Syndrome

[CIS-PIDD]

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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