SEARCH:   Advanced

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 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 Citation Map 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 Google Scholar Articles by Colombat, M. Articles by Delfau-Larue, M.-H. PubMed PubMed Citation Articles by Colombat, M. Articles by Delfau-Larue, M.-H. Related Collections Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  NEOPLASIA Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor Magali Colombat1, Hervé Mal2, Christiane Copie-Bergman35, Jacques Diebold6, Diane Damotte7, Patrice Callard1, Michel Fournier2, Jean-Pierre Farcet5,8, Marc Stern9, and Marie-Hélène Delfau-Larue4,5,8 1 Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Tenon, Service d'anatomie pathologique, Paris; 2 APHP, Hôpital Bichat, Service de pneumologie, Paris; 3 APHP, Hôpital Henri Mondor, Département de Pathologie, Créteil; 4 Inserm, U841 équipe 9, Créteil; 5 Université Paris 12, Faculté de Médecine, Créteil; 6 APHP, Hôtel Dieu, Service d'anatomie pathologique, Paris; 7 APHP, Hôpital Européen Georges Pompidou, Service d'anatomie pathologique, Paris; 8 APHP, Hôpital Henri Mondor, Service d'immunologie biologique, Créteil; and 9 Hôpital Foch, Service de pneumologie, Suresnes, France 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 3 patients with cystic lung LCDD. Histologic examination of the explanted lungs showed diffuse nonamyloid light chain deposits associated with a mild lymphoid infiltrate composed of aggregates of small CD20+, CD5–, CD10– B lymphocytes reminiscent of bronchus-associated lymphoid tissue. Using polymerase chain reaction (PCR), we identified a dominant B-cell clone in the lung in the 3 studied patients. The clonal expansion of each patient shared an unmutated antigen receptor variable region sequence characterized by the use of IGHV4-34 and IGKV1 subgroups 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 biologic 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?

	Copyright © 2008 by American Society of Hematology         Online ISSN: 1528-0020