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Blood, 1 September 2008, Vol. 112, No. 5, pp. 2004-2012. Prepublished online as a Blood First Edition Paper on May 15, 2008; DOI 10.1182/blood-2007-11-123596. This Article Full Text (PDF) All Versions of this Article: blood-2007-11-123596v1 112/5/2004    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Colombat, M. Articles by Delfau-Larue, M.-H. Search for Related Content PubMed PubMed Citation Articles by Colombat, M. Articles by Delfau-Larue, M.-H. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted November 14, 2007 Accepted February 24, 2008 Primary cystic lung light chain deposition disease : a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor Magali Colombat, Herve Mal, Christiane Copie-Bergman, Jacques Diebold, Diane Damotte, Patrice Callard, Michel Fournier, Jean-Pierre Farcet, Marc Stern, and Marie-Helene Delfau-Larue* APHP, Hopital Tenon, Service d'anatomie pathologique, 7520 Paris, France APHP, Hopital Bichat, Service de pneumologie, 75018 Paris, France APHP, Hopital Henri Mondor, Departement de Pathologie, Creteil, F-9400, France APHP, Hotel Dieu, Service d'anatomie pathologique, 75004, Paris, France APHP, Hopital Europeen Georges Pompidou, Service d'anatomie pathologique, 75015 Paris, France AP-HP, Hopital Henri Mondor, Service d'immunologie biologique, INSERM, U841 equipe 9, Universite Paris 12, Faculte de Medecine, Creteil, F-9400, France Hopital Foch, Service de pneumologie, 92151 Suresnes, France * Corresponding author; email: marie-helene.delfau{at}hmn.aphp.fr. We have recently described a new form of light chain deposition disease (LCDD) presenting as a severe cystic lung disorder requiring lung transplantation. There was no bone marrow plasma cell proliferation. Because of the absence of disease recurrence after bilateral lung transplantation and of serum free light chain ratio normalization after the procedure, we hypothesized that monoclonal light chain synthesis occurred within the lung. The aim of this study was to look for the monoclonal B-cell component in three patients with cystic lung LCDD. Histological examination of the explanted lungs showed diffuse non-amyloid light chain deposits associated with a mild lymphoid infiltrate mainly composed of small CD20+, CD5-, CD10- B lymphocytes arranged in nodules reminiscent of bronchus-associated lymphoid tissue. Using PCR, we identified a dominant B-cell clone in the lung in the three studied patients. The clonal expansion of each patient shared a unmutated antigen receptor variable region sequence characterized by the use of VH4-34 and VK1 gene family with heavy and light chain CDR3 sequences of more than 80% amino acid identity, a feature evocative of an antigen-driven process. Combined with clinical and biological data, our results strongly argue for a new antigen-driven primary pulmonary lymphoproliferative disorder. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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