[CIS PIDD] [cis-pidd] Adult CVID, Recurrent Shingles

Wed Apr 26 16:38:14 EDT 2017

Thank you Marc and Bodo.  The patient is 66yo.  She is in very good health in general with no evidence of malignancy.  No known risk factors:  never smoked, no known exposures.  JC

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Wednesday, April 26, 2017 11:49 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Adult CVID, Recurrent Shingles (EXTERNAL EMAIL)

... Or a sign of an undiagnosed malignancy.
How old is your patient? Any risk factors for cancer?
Yours, Bodo

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Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
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Breisacherstraße 115, 79106 Freiburg
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Von: <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Wednesday 26 April 2017 15:56
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: Re: [cis-pidd] Adult CVID, Recurrent Shingles

Hi Joe,

Personally I think that H zoster is very (very)  rare in CVID pts on Ig therapy; my first impulse is to validate it with cultures.

Charlotte Cunningham-Rundles

On Apr 25, 2017, at 10:33 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hi Joe,

I have not attempted, but most of these are in general HSV-1 recurrent whitlow, HSV-2 buttock/lower back lesions - or other similar site often in typical locations

Most of the breakthroughs are in HSV caused by
- either patient forgetting to take the medication regularly or
- by a temporal drug resistant mutant clone which however has a lesser fit for host and thus does not recur and does not establish dormancy in the ganglia.

So
- a single breakthrough: do nothing, emphasize regularity of medication
- recurrent breakthroughs: establish dg and if HSV, is the dose of valaciclovir large enough (up to 3g/daily)?
- if HSV, breakthroughs despite 3g/d OR if VZV
a) is CVID truly CVID? Highly frequent shingles makes me think of CID, a few occasional recurrences one does see in VZV and "CVID"
b) and in the case of HSV, is there even an impaired IFNg response, see Mihai Netea's group's article recently in JACI? However, patient would need to have to conditions, not exactly the Occam's razor

ATB,

Mikko Seppänen

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 25.4.2017 kello 22.03:
Colleagues:

I just started following an adult woman who was previously diagnosed with CVID.  She is doing very well with regards to respiratory and GI symptoms on weekly SCIg infusions.

However, she has experienced recurrent "shingles" and recently broke through valacyclovir prophylaxis.  The lesions have not been PCR'd for VZV.  She was told by another MD that a VZV vaccine may help.

I am a bit hesitant because she has a good VZV-specific lymphoproliferative response.

Assuming these are actually recurrent VZV outbreaks, has anyone noted an improvement in frequency or severity after administration of VZV vaccine?

Thank you for your time.

Joe Church
Children's Hospital Los Angeles

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