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

[Mel.Berger at cslbehring.com]

Regardless of whether it is caused by the drugs singly or in combination, you have a patient who is having enough problems to have had multiple sinus X-rays and CT scans, low total IgG, and (we are assuming) non-protective antibody titers even after immunization. If that assumption turns out to be the fact, I would definitely have her on IgG replacement. Further, I would evaluate her chest carefully. If there is any evidence of bronchial or parenchymal disease, that would provide a further push towards IgG replacement.



Mel Berger

________________________________
From: dmvascon at usp.br [dmvascon at usp.br]
Sent: Monday, April 29, 2013 1:49 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Tuberous Sclerosis and CVID?

Dear Zach and all

I agree with you about the risk of hypogammaglobulinemia induced by anticonvulsants (mainly hydantoinate and carbamazepine).
I've seen several due to hydantoin (only one recovered Ig production after 18 months of withdraw), but none with the drugs used by your patient.

I can assume that there is a potential risk of hypogammaglobulinemia due to sirolimus, due to the fact that it acts on mTOR, decreasing IL-2 actions in T and B cells (but I've never seen such a reaction).

Thinking in a practical way I would start IVIg replacement. Making a parallel with Rasmussen's syndrome it is possible that the convulsive disorder of your patient improves on IVIg (only time will answer to this question).

Best,

Dewton

Dewton de Moraes Vasconcelos, M.D.; Ph.D.
University of São Paulo School of Medicine
________________________________
De: "Zachary D. Jacobs, MD" <zjacobs.md at gmail.com>
Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>
Enviadas: Segunda-feira, 29 de Abril de 2013 13:48:32
Assunto: [cis-pidd] Tuberous Sclerosis and CVID?


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

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