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IgG1 Subclass Restriction and Biochemical Peculiarities of Monoclonal Immunoglobulins in Scleromyxedema

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  • معلومة اضافية
    • Contributors:
      CHU Saint-Antoine AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU); Centre de Recherche Saint-Antoine (CRSA); Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU); Service de Dermatologie AP-HP Hôpital Saint-Louis; Hopital Saint-Louis AP-HP (AP-HP); Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Service de dermatologie Mondor; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12); Epidemiology in Dermatology and Evaluation in Therapeutics (EpiDermE); Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12); Groupe Hospitalier du Havre Hôpital Jacques Monod (MONTIVILLIERS) (GHH); Hôpital Saint Vincent de Paul de Lille; Groupement des Hôpitaux de l'Institut Catholique de Lille (GHICL); Université catholique de Lille (UCL)-Université catholique de Lille (UCL); Centre Hospitalier de Dax; Hôpital de la Cavale Blanche - CHRU Brest (CHU - BREST ); Hôpital Provo; CHU Pitié-Salpêtrière AP-HP
    • بيانات النشر:
      HAL CCSD
      Clinical Laboratory Publications
    • الموضوع:
      2021
    • Collection:
      Inserm: HAL (Institut national de la santé et de la recherche médicale)
    • نبذة مختصرة :
      International audience ; 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.
    • Relation:
      info:eu-repo/semantics/altIdentifier/pmid/33739037; inserm-03175493; https://inserm.hal.science/inserm-03175493; https://inserm.hal.science/inserm-03175493/document; https://inserm.hal.science/inserm-03175493/file/%23200605%20Petersen%20et%20al%202020%20manuscript%20revised1_DHO%20pa%20final.pdf; PUBMED: 33739037
    • الرقم المعرف:
      10.7754/Clin.Lab.2020.200605
    • Rights:
      info:eu-repo/semantics/OpenAccess
    • الرقم المعرف:
      edsbas.6E620DDD