[CIS PIDD] some help from Chile

[patricia roessler vergara]

Dear Christina:
Thank you for your answer
Me and the patient would be very happy to send you samples, so tell me how
can we proceed for that.
About the 50% of your patients that you finally catalog as CVID....in what
do you base the diagnosis, only because they maintain altered memory B
cells after two years of rituxan? or because they have specific antibodies
alterations. If they have low response to vaccination could also be do to
rituxan or is more specific of CVID.
I found that my patient had specific anti pneumococcal antibodies and they
were all low (pre-vaccination), I am waiting for the post vaccination ones

Thanks
Patricia

If you would like to send me her samples, I would be happy to include your
patient.
2012/7/5 Christina Price <christinachia1 at gmail.com>


> Patricia Roessler

>

>

>

> I have been collecting patients just like yours here at Yale- patients

> with autoimmune conditions who received Rituximab, were

> hypogammaglobulinemic, normal peripheral B cell number but low

> switched/unswitched memory B cells, presenting with frequent infections,

> sometimes refractory to antibiotics and required IVIG.  About half of the

> patients have been given a diagnosis of CVID 1a. The other half, we are

> waiting to see how they turn out. I give the patients at least 2 years to

> fully recover their memory compartment.

>

>

> What was her IgG level prior to Rituximab? In these patients that receive

> RTX, the pre Rituximab treatment immune work up is critical.

>

>

>

> Regarding your questions

>

> 1. I don't think the answer is clear. Yes I think these finding CAN be

> secondary to Rituximab. The long term consequences of RTX are unknown. Your

> patient has normal IgM and IgA levels, so does not fit the traditional

> criteria for CVID diagnosis. However, her last dose of RTX was more than 2

> years ago. I agree with you that autoimmunity and immune deficiency share

> immune dysregulation. I am doing further work on my patients like this. If

> you would like to send me her samples, I would be happy to include your

> patient.

>

>

> 2. The literature tells us that patients with low IgG at onset of RTX have

> more infection risk. Before your patients receives more RTX, I would wait

> for the pneumococcal results. If they are low, vaccinate with

> polysaccharide and conjugate vaccines. I would also check her vaccine

> titers to Hib, Hepatitis B, tetanus, and others and vaccinate accordingly

> BEFORE rituximab is given.

>

>

>

> 3. Unless she is seriously ill, it sounds like you have time to wait for

> the pneumococcals. I would give IVIG. Your patient clinically has frequent

> infections. While the bladder infections are common in MS, I have also seen

> CVID patients with frequent UTIs. We reported on a young female with

> myasthenia gravis with bilateral mastoiditis that responded very well to

> IVIG.

>

> Am J Otolaryngol. <http://www.ncbi.nlm.nih.gov/pubmed/22361345#> 2012 Feb

> 21.

>  Use of intravenous immunoglobulin to treat chronic bilateral

> otomastoiditis in the setting of rituximab induced hypogammaglobulinemia.

> Otremba MD<http://www.ncbi.nlm.nih.gov/pubmed?term=Otremba%20MD%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>

> , Adam SI<http://www.ncbi.nlm.nih.gov/pubmed?term=Adam%20SI%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>

> , Price CC<http://www.ncbi.nlm.nih.gov/pubmed?term=Price%20CC%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>

> , Hohuan D<http://www.ncbi.nlm.nih.gov/pubmed?term=Hohuan%20D%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>

> , Kveton JF<http://www.ncbi.nlm.nih.gov/pubmed?term=Kveton%20JF%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>

> .

>  Source

>

> Departments of Surgery, Section of Otolaryngology, Head, and Neck

> Surgery, Yale University School of Medicine, New Haven, CT.

>

>

> http://www.ncbi.nlm.nih.gov/pubmed/22361345

>

>

> Hope this is helpful. I have been looking at this issue for the last 2

> years as your clinical problem has come up several times. It has become a

> research interest, so I hope to be able to provide more answers in the

> future.

>

>

> Best of luck,

>

>

> Christina

>

>

>

> Christina C. Price, MD

>

> Yale University School of Medicine

>

> Instructor

>

> Allergy and Clinical Immunology

>

>

>

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