[CIS PIDD] [cis-pidd] B-cell subsets

[CIS-PIDD]

Could it be some form of plasma cell deficiency (like mutation of
SEC61A1 (protein transporter in ER) presented by Bodo Grimbacher on ESID
2016)?
Reported patients had only infections but it seems to me that in
this case % of B cell subsets were normal.
Milica M.  

Il 02.02.2017
02:18 CIS-PIDD ha scritto: 

> They are "low risk" CVIDs with infections
only; no lymphoproliferative disorders, no GI symptoms, no autoimmune
issues (?yet). JC 
> 
> FROM: CIS-PIDD
[mailto:cis-pidd at lists.clinimmsoc.org] 
> SENT: Wednesday, February 01,
2017 5:16 PM
> TO: CIS-PIDD
> SUBJECT: RE: [cis-pidd] B-cell subsets 
>

> Hi Joe,
> 
> Do your patients have only infections or also other
non-infectious manifestations of CVID?
> 
> Best,
> Milica Mitrevski 
>

> Il 02.02.2017 01:07 CIS-PIDD ha scritto: 
> 
>> Yes, very normal
numbers. JC 
>> 
>> FROM: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org
[1]] 
>> SENT: Wednesday, February 01, 2017 3:51 PM
>> TO: CIS-PIDD
>>
SUBJECT: Re: [cis-pidd] B-cell subsets 
>> 
>> Hi Joe, 
>> 
>> Do the
patients have normal numbers of B cells? 
>> 
>> Best, 
>> 
>> Jordan

>> 
>> FROM: CIS-PIDD 
>> REPLY-TO: CIS-PIDD 
>> DATE: Wednesday,
February 1, 2017 at 2:34 PM
>> TO: CIS-PIDD 
>> SUBJECT: [cis-pidd]
B-cell subsets 
>> 
>> Colleagues:
>> 
>> Several of my CVID patients
have displayed phenotypes similar to the following: 
>> 
>> · CD19+ IgD+
CD27- (naïve) 20% 
>> 
>> · CD19+ IgD+ CD27+ (memory) 48% 
>> 
>> ·
CD19+ IgD- CD27+ (switched memory 31% 
>> 
>> Are there specific "CVID"
genotypes that are likely to present with this pattern? 
>> 
>> Joe
Church 
>> 
>> Children's Hospital Los Angeles 
>> 
>>
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> 
> --
> 
> Milica Mitrevski, MD, PhD Clinical Immunology and
Allergy Specialist Sapienza University of Rome Department of Clinical
Medicine Viale dell'Università 37, 00185 Rome, Italy tel: +39 06
49972036 +39 349 5622376 mail: mitmilica at tiscali.it [14] 
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