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

Fri Feb 3 11:27:04 EST 2017

How can we do the testing for this new SEC61 deficiency. I have couple of patients who have significant hypogam with normal B cells and switched memory B cells.

Anilkumar Katta, M.D.

Clinical Asst. Professor of Medicine, Tufts University School of Medicine,

Department of Allergy and Immunology, Lahey Hospital and Medical Center,

31 Mall Rd., Burlington, MA - 01805.

Phone: 781-744-8442.

Fax: 781-744-3442.

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Sent: Thursday, February 02, 2017 4:46 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] B-cell subsets

One molecular explanation for “infection only" CVID patients  with normal B cell subsets could be the new Sec61 deficiency, unpublished, but I showed the data at ESID 2017: Pure plasma cell defect…

Yours, Bodo

****************************************

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

Scientific-Director

CCI-Center for Chronic Immunodeficiency

UNIVERSITÄTSKLINIKUM FREIBURG

Tel.: 0761 270-77731  Fax: -77744

Breisacherstraße 115, 79106 Freiburg

bodo.grimbacher at uniklinik-freiburg.de 

www.uniklinik-freiburg.de/cci

and 

Consultant Immunologist

Institute of Immunity & Transplantation

Dept of Immunology

Royal Free Hospital

UNIVERSITY COLLEGE LONDON

Pond Street

London NW3 2QG

b.grimbacher at ucl.ac.uk

www.centreforimmunodeficiency.com

Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
Datum: Thursday 2 February 2017 15:37
An: CIS-PIDD <cis-pidd at lyris.dundee.net>
Betreff: RE: [cis-pidd] B-cell subsets

Thanks, Mikko, Klaus.

The patients are remarkably typical for “uncomplicated” CVID:  normal total B-cells, IgGs 200-500, poor antibodies following Pneumovax.  

I will look for “accessory” issues.

JC

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Sent: Wednesday, February 01, 2017 9:58 PM
To: CIS-PIDD
Subject: AW: [cis-pidd] B-cell subsets

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

www.uniklinik-freiburg.de/cci[uniklinik-freiburg.de] <https://urldefense.proofpoint.com/v2/url?u=http-3A__www.uniklinik-2Dfreiburg.de_cci&d=CwMGaQ&c=mLNPgKHx4J-iKlzX2GMoHFvr3_xqSCZA8BjFUsFWPt4&r=MdH0U6P9_jn42qvKYuO2TxkOeHSKWGSWrWAwp5482mI&m=_hraO5ThUodHGytul2yx4qYWuLvpWusLLcC7hY7Rygc&s=Y6Wxqlhm2qDb-TErGY1qpUDkbam7chHQ64YkVT4x3gc&e=> 

________________________________

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[dx.doi.org] <https://urldefense.proofpoint.com/v2/url?u=https-3A__dx.doi.org_10.1007_s10875-2D016-2D0264-2D7&d=CwMGaQ&c=mLNPgKHx4J-iKlzX2GMoHFvr3_xqSCZA8BjFUsFWPt4&r=MdH0U6P9_jn42qvKYuO2TxkOeHSKWGSWrWAwp5482mI&m=_hraO5ThUodHGytul2yx4qYWuLvpWusLLcC7hY7Rygc&s=G57rFPOdv3SARV2UJBybyAJ_3v1K3zwhTDYwckaKHYA&e=> 

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