[CIS PIDD] [cis-pidd] IgG rash in CVID patient

Thu Nov 30 12:23:18 EST 2017

Thanks Richard.
I don't think there were any real trough levels checked unfortunately as the patient was being cared for by oncology and they were not monitoring this all that closely and the patient was not very compliant with treatment, so dosing was erratic.  The levels that are available are not very useful during the time she was treated but those that are available were all still very low (ie 
| Component
 Latest Ref Rng & Units | 9/14/2015 | 9/25/2015 | 10/23/2015 | 12/23/2015 |
| IgG
 635 - 1741 MG/DL | 139 (L) (off IgG for several months) | 128 (L) (off IVIG for several months) | 81 (L) (after one dose of IVIG) | 299 (L) (after ~2 doses of IVIG and then stopping IVIG after 10/23/15 infusion due to development of rash) |

As for her dose, it looks like she was receiving 40grams IV (which was about 0.4 grams/kg I think).
Thank you for your thoughts/recommendations.
I will see if the pathologists/lab can do immunofluorescence studies of the biopsy.
-Shayna

      From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
 To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
 Sent: Thursday, November 30, 2017 10:42 AM
 Subject: Re: [cis-pidd] IgG rash in CVID patient

The suggestions to use IV products subq and to consider complement deficiency and immune complexes are good ones. You didn't tell us what dose the patient was receiving when she was getting IGIV or what her trough level was. I couple of thoughts come to mind:1. The excipients for Privigen (proline) and Gammagard (glycine) are different implying that it is the IgG and that it is unlikely that a change in product will make a difference. One could argue that the rash is being caused by trace contaminants but I doubt it.2. Despite my comment above, I have used Carimune NF subq at 16% without a problem.3. If the patient does have a complement deficiency, I don't know what could be done differently.4. Although I have great respect for Marianna, I don't think it will be possible to desensitize to IG as though it was a small molecule.5. If the rash is being caused by deposition of immune complexes, that could be confirmed by immunofluorescence studies of the biopsy. If there are immune complexes, then I would consider using high dose IVIG in an effort to capture the antigenic load. The first infusion is likely to result in rash but if you keep the level high, the rash may not continue. Actually, if you are in high enough antibody excess, immune complexes may not be deposited. If you think that the antigens in the immune complexes are bacterial, you might treat with a several week course of a broad spectrum antibiotic before that first dose of IGIV.
Richard WassermanDallas
On Thu, Nov 30, 2017 at 9:23 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:

Hi Shayna, You may consider checking complement levels. Sometimes patients with Complement deficiency form immune complexes (IVIG and circulating antigens) and fail to clear those due to complement deficiency. A nice abstract last year during CIS meeting described a similar case, and smaller IVIG dose seemed to help. https://cis.confex.com/cis/ 2017/webprogram/Paper5106.html regards,Joud Joud Hajjar MD, MS.Assistant Professor of MedicineService Chief, Adult Allergy and ImmunologyBaylor College of Medicine and Texas Children's HospitalSection of Immunology, Allergy & Rheumatologyjoud.hajjar at bcm.edu  From: <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org >
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Thursday, November 30, 2017 at 7:18 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re: [cis-pidd] IgG rash in CVID patient ***CAUTION:*** This email is not from a BCM Source. Only click links or open attachments you know are safe.Hi All, I am treating a 39 you female with ITP and CVID previously diagnosed by oncology.  Prior to starting IVIG, she had nondetectable immunoglobulins (ie. IgA <10, IgM <20, IgG <75).   I was asked to see her after she had been off of IVIG for >1 year and required multiple admissions to the hospital for sinopulmonary infections (~every 2-3 months) while being off of IVIG.  Her IVIG was stopped since Nov 2015 due to development of worsening rashes while on both Privigen and Gammagard.  Her rashes were diffuse popular extremely pruritic rashes that eventually became painful as well.  She required steroids for treatment and the rashes eventually resolved but have left permanent hyperpigmentation that the patient is not happy about.  When I saw her recently, I suggested a trial of Hizentra weekly, as I hoped that smaller doses SQ weekly might prevent the rashes from occurring and allowing her to received her needed IgG replacement.  The patient tolerated the first 2-3 infusions without any problems, but over the past 2 weeks has now again developed extremely pruritic papules/patches diffusely on her legs, arms, abd, back, and neck.  However, she has not gotten sick nor been hospitalized since starting Hizentra, which is very good for her.   Biopsy of one of the lesions showed likely drug reaction (see below for official path results). Skin with Chronic Interface Dermatitis and Chronic Perivascular Dermatitis
(Superficial and Deep). 
Comment: The surface of the skin shows hyperkeratosis. There is a chronic 
interface lymphocytic dermatitis with spongiosis. Superficially and also in the 
deeper dermis, there is a pronounced chronic inflammatory perivascular 
infiltrate composed of lymphocytes. The findings favor an allergic/ 
hypersensitivity response or possible drug reaction.    So, I'm wondering if anyone has any recommendations on how to proceed?  She clearly ideally needs IgG replacement, but I'm not sure how to get it to her.Thanks!  -Shayna Burke, MDAllergy and Asthma Family Care/St Francis Hospital and Medical CenterConnecticut   ---You are currently subscribed to cis-pidd as:shaynaburke at yahoo.com.To unsubscribe click here:http://cts.dundee.net/u?id= 96396884. 0c351f975bf8c3cf588a002ca42514 89&n=T&l=cis-pidd&o=4681117(It may be necessary to cut and paste the above URL if the line is broken)or send a blank email toleave-4681117-96396884. 0c351f975bf8c3cf588a002ca42514 89 at lyris.dundee.net ---You are currently subscribed to cis-pidd as: joud.hajjar at bcm.edu.To unsubscribe click here: http://cts.dundee.net/u?id= 96396690. 01670be70beac432bf57127b486e91 65&n=T&l=cis-pidd&o=4681976(It may be necessary to cut and paste the above URL if the line is broken)or send a blank email to leave-4681976-96396690. 01670be70beac432bf57127b486e91 65 at lyris.dundee.net ---  You are currently subscribed to cis-pidd as: drrichwasserman at gmail.com.  To unsubscribe click here: http://cts.dundee.net/u?id= 96396499. 3449c9fd54f25f9fa0e022cb002b8c 1e&n=T&l=cis-pidd&o=4682168  (It may be necessary to cut and paste the above URL if the line is broken)  or send a blank email to leave-4682168-96396499. 3449c9fd54f25f9fa0e022cb002b8c 1e at lyris.dundee.net

-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211 ---  You are currently subscribed to cis-pidd as: shaynaburke at yahoo.com.  To unsubscribe click here: http://cts.dundee.net/u?id=96396884.0c351f975bf8c3cf588a002ca4251489&n=T&l=cis-pidd&o=4682197  (It may be necessary to cut and paste the above URL if the line is broken)  or send a blank email to leave-4682197-96396884.0c351f975bf8c3cf588a002ca4251489 at lyris.dundee.net

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