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

[dmvascon at usp.br]

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 
----- Mensagem original -----


> 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

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