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Journal articles
IgG1 Subclass Restriction and Biochemical Peculiarities of Monoclonal Immunoglobulins in Scleromyxedema
Tania Petersen
1, 2
Thibault Mahévas
3
Pauline Cannet
1, 2
Jean-David Bouaziz
4
Camille Hua
5, 6
Charles Zarnitsky
7
Philippe Modiano
8
François Lifermann
9
Bruno Sassolas
10
Olivier Carpentier
11
Arsène Mékinian
3
Laurent Garderet
12
Pierre Aucouturier
1, 2
Yannick Chantran
1, 2
Tania Petersen 1, 2
Author
Thibault Mahévas 3
Author
Pauline Cannet 1, 2
Author
Jean-David Bouaziz 4
Author
Camille Hua 5, 6
Author
Charles Zarnitsky 7
Author
Philippe Modiano 8
Author
François Lifermann 9
Author
Bruno Sassolas 10
Author
Olivier Carpentier 11
Author
Arsène Mékinian 3
Author
Laurent Garderet 12
Author
Pierre Aucouturier 1, 2
Author
Yannick Chantran 1, 2
Author
1
Service d'immunologie et hématologies biologiques [Saint-Antoine] (184 rue du Faubourg Saint Antoine 75012 Paris - France)
- SU - Sorbonne Université (21 rue de l’École de médecine - 75006 Paris - France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- CHU Saint-Antoine [AP-HP] (184, rue du Faubourg Saint-Antoine
75571 Paris cedex 12 - France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- Sorbonne Université (France)
2
CR Saint-Antoine - Centre de Recherche Saint-Antoine (France)
- CHU Saint-Antoine [AP-HP] (184, rue du Faubourg Saint-Antoine
75571 Paris cedex 12 - France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- Sorbonne Université (France)
- INSERM - Institut National de la Santé et de la Recherche Médicale : U938 (101, rue de Tolbiac, 75013 Paris - France)
- SU - Sorbonne Université (21 rue de l’École de médecine - 75006 Paris - France)
3
Service de médecine interne [Saint-Antoine] (184, rue du Faubourg Saint-Antoine 75571 PARIS Cedex 12 - France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- SU - Sorbonne Université (21 rue de l’École de médecine - 75006 Paris - France)
- CHU Saint-Antoine [AP-HP] (184, rue du Faubourg Saint-Antoine
75571 Paris cedex 12 - France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- Sorbonne Université (France)
4
Service de Dermatologie [AP-HP Hôpital Saint-Louis] (1 avenue Claude-Vellefaux, 75475 Paris Cedex 10. - France)
- AP-HP - Hopital Saint-Louis [AP-HP] (1 Avenue Claude Vellefaux, 75010 Paris - France)
5
Service de dermatologie [Mondor] (France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- Hôpital Henri Mondor (France)
- UPEC UP12 - Université Paris-Est Créteil Val-de-Marne - Paris 12 (61 avenue du Général de Gaulle - 94010 Créteil cedex - France)
6
EpiDermE - Epidemiology in Dermatology and Evaluation in Therapeutics (Université Paris Est Créteil - Campus Centre de Créteil
61 Avenue du Général de Gaulle 94010 Créteil cedex - France)
- UPEC UP12 - Université Paris-Est Créteil Val-de-Marne - Paris 12 : EA7379 (61 avenue du Général de Gaulle - 94010 Créteil cedex - France)
7
GHH - Groupe Hospitalier du Havre Hôpital Jacques Monod (MONTIVILLIERS) (29 Avenue Pierre Mendès
76290 Montivilliers - France)
8
Hôpital Saint Vincent de Paul de Lille (Hôpital Saint Vincent de Paul
Boulevard de Belfort - BP 387
59020 Lille Cedex - France)
9
Centre Hospitalier de Dax (France)
10
CHU - BREST - Hôpital de la Cavale Blanche - CHRU Brest (Boulevard Tanguy Prigent 29609 Brest Cedex - France)
11
Hôpital Provo (France)
12
Service d'Hématologie Clinique [CHU Pitié-Salpêtrière] (France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
- CHU Pitié-Salpêtrière [AP-HP] (47-83 Boulevard de l'Hôpital, 75013 Paris - France)
- Sorbonne Université (France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
Abstract : Background: Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were reported in case reports or short series, such as IgGλ over-representation, cationic migration, and partial deletion. Methods: Monoclonal immunoglobulins (Ig) from the serum of 12 consecutive patients diagnosed with scleromyxedema (SME) were analyzed using electrophoretic and immunoblotting techniques. Results: All monoclonal Ig from 12 SME were of IgG1 subclass, with an overrepresentation of the lambda-type light chain and a cationic mobility on standard zone electrophoresis, as compared with 21 cases of monoclonal gammopathy of undetermined significance (MGUS) of IgG1 subclass. Reactivity with specific monoclonal antibodies demonstrated no evident deletion of the heavy chain constant domains, which was also confirmed by analysis of Ig heavy chain molecular weight on a purified monoclonal component from one case. Conclusions: Significant isotype restriction of both heavy and light chains, and peculiar biochemical properties suggest that monoclonal Ig might be involved in pathophysiological events of SME.
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Journal articles
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https://www.hal.inserm.fr/inserm-03175493
Contributor : Pierre Aucouturier <>
Submitted on : Saturday, March 20, 2021 - 11:36:45 AM
Last modification on : Friday, June 4, 2021 - 11:24:03 AM
Contributor : Pierre Aucouturier <>
Submitted on : Saturday, March 20, 2021 - 11:36:45 AM
Last modification on : Friday, June 4, 2021 - 11:24:03 AM
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Identifiers
- HAL Id : inserm-03175493, version 1
- DOI : 10.7754/Clin.Lab.2020.200605
- PUBMED : 33739037
Collections
INSERM | U938 | UPEC-UPEM | UPEC | SORBONNE-UNIVERSITE | SU-MEDECINE | EPIDERME | SU-TI
Citation
Tania Petersen, Thibault Mahévas, Pauline Cannet, Jean-David Bouaziz, Camille Hua, et al.. IgG1 Subclass Restriction and Biochemical Peculiarities of Monoclonal Immunoglobulins in Scleromyxedema. Clinical Laboratory, Clinical Laboratory Publications, 2021, 67 (03), Online ahead of print. ⟨10.7754/Clin.Lab.2020.200605⟩. ⟨inserm-03175493⟩
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