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

Thu Feb 2 00:58:15 EST 2017

thank you Mikko :-)

I agree with Mikko, this phenotype would make me search for alternative explanations although there are CVID patients with this phenotype including possibly some TACI patients where there is normal memory formation although rarely so strongly shifted that direction.

Anything common in the history of your patients, Joe?

how deficient are they in their antibodies? antibody responses?

greetings

Klaus

Prof. Dr. med. Klaus Warnatz

MEDICAL CENTER – UNIVERSITY OF FREIBURG
Center for Chronic Immunodeficiency – CCI
at the Center for Translational Cell Research
Department of Rheumatology and Clinical Immunology

Breisacher Str. 115, 79106 Freiburg, Germany
Tel. +49 761 270 77640 / FAX -77600 / Pager 12-7100
klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>

www.uniklinik-freiburg.de/cci

________________________________
Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Gesendet: Donnerstag, 2. Februar 2017 06:39
An: CIS-PIDD
Betreff: Re: [cis-pidd] B-cell subsets

Hi Joe,

I have seen this in 1/appr. 150 patients with primary CVID.

However, a common finding in secondary, steroid-induced hypogamma, like Klaus Warnatz and colleagues have shown in their brilliant and clinically extremely important study:

Secondary Antibody Deficiency in Glucocorticoid Therapy Clearly Differs from Primary Antibody Deficiency.
Wirsum C, et al. J Clin Immunol. 2016.

J Clin Immunol. 2016 May;36(4):406-12. doi: 10.1007/s10875-016-0264-7. Epub 2016 Mar 15.

Abstract

PURPOSE: The aim of this study was to identify characteristics of hypogammaglobulinemia secondary to glucocorticoid therapy and their value in the differential diagnosis to primary forms of antibody deficiency.

METHODS: We investigated prevalence and character of hypogammaglobulinemia in a cohort of 36 patients with giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) on glucocorticoid therapy in comparison to a gender- and age-matched cohort of hospital controls. We therefore determined serum immunoglobulin levels as well as B- and T cell-subsets in the peripheral blood of all participants. In addition, prior serum immunoglobulin levels and clinical data of the GCA and PMR patients were extracted from the electronic patient data-base.

RESULTS: 21/36 GCA/PMR patients on glucocorticoid treatment developed antibody deficiency. In 19 patients this included IgG and in 13 patients IgG was the only affected isotype. The reduction of IgG was persistent in nearly 50 % of these patients during the observed period. GCA/PMR patients had reduced circulating naive and transitional B cells (p = 0.0043 and p = 0.0002 respectively) while IgM, IgG and IgA memory B cells were preserved. Amongst T-cell subsets, we found a reduction of CD4 memory T cells (p < 0.0001), CD4 regulatory T cells (p = 0.0002) and few CD8 memory T-cell subtypes.

CONCLUSION: Persistent humoral immunodeficiency occurs in about a quarter of GCA/PMR patients under glucocorticoid therapy. Because most patients have isolated IgG deficiency, preserved IgA production and class-switched memory B cells, by these markers this form of secondary hypogammaglobulinemia can be clearly distinguished from common variable immunodeficiency (CVID).

PMID
 26980224 [PubMed - in process]
Full text
Full text at journal site<https://dx.doi.org/10.1007/s10875-016-0264-7>

ATB

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

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 2.2.2017 kello 1.51:

Hi Joe,
Do the patients have normal numbers of B cells?
Best,
Jordan

From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Wednesday, February 1, 2017 at 2:34 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
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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