[CIS PIDD] [cis-pidd] Tuberous Sclerosis and CVID?

[Zachary D. Jacobs, MD]

Hello all,

I was sent for immune evaluation via pulmonology a 54 year-old woman with
Tuberous Sclerosis and secondary lymphangioleimyomatosis (LAM) and seizure
disorder.  She has been having recurrent X-ray and CT proven sinopulmonary
infections over the past 12 months.  She is on sirolimus for LAM and has
been on this for five years.  She is on four anti-convulsants (divalproex,
keppra, lamictal and zonisamide) for control of her seizures, and has been
on all of them for several years.

Her IgG is 270 mg/dl, IgA is 34 mg/dl, and IgM is 50 mg/dl.  S. pneumo
specific antibody panel showed concentrations above 1.3 mcg/mL in only two
of the 23 serotypes, at 2.4 and 3.1.  The remainder were not even close to
1.3.  Tetanus antibody was low-normal at 0.37 IU/mL.  I am awaiting
post-vaccination studies but let’s assume for the sake of argument there is
no significant response.  CMP and CBC were normal.  Flow cytometry of basic
lymphocyte subsets was normal.

I wanted to gauge the community’s thoughts as to whether this is primary or
secondary.  I am leaning towards primary CVID.  But, she is on several
anti-convulsants, which I know can cause antibody deficiency but have never
personally seen.  I do not think the sirolimus could cause these findings,
right?  If you think it is secondary, how would you manage it other than
starting immunologlobulin replacement?  There are not very many
alternatives for seizure control that she is not already on.

Thanks as always for the input.

Zach
-- 
Zachary D. Jacobs, M.D.

The Center for Allergy & Immunology

Saint Luke’s Physician Partners
Medical Plaza II
4330 Wornall, Suite 40
Kansas City, MO 64111

Ph: 816.531.0930
Fax: 816.753.2671

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