ArticleBlood2008
Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor.
Article in Blood, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 39 citations in OpenAlex.
- Light Chain Deposition Disease - presenting with Cystic Pulmonary Disorder as primary manifestation - A case report.Respiratory medicine case reports · 2026Article
- Isolated Pulmonary Light Chain Deposition Disease.European journal of case reports in internal medicine · 2026Article
- Pulmonary light chain deposition disease: Case series and review of the literature.Radiology case reports · 2024Article
- Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.Respiratory research · 2024Article
- Dyspnea and cough in a 68-year-old female with light chain deposition disease.Respiratory medicine case reports · 2023Article
- Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management.Diagnostics (Basel, Switzerland) · 2021Review
- Pulmonary light chain deposition disease: a case series and literature review.Annals of translational medicine · 2020Article
- Diffuse Cystic Lung Disease. Part II.American journal of respiratory and critical care medicine · 2015Review
- Pulmonary langerhans cell histiocytosis.Orphanet journal of rare diseases · 2012Review
- Extraosseus plasmacytoma of the pharynx with localized light chain deposition. Case report.Pathology oncology research : POR · 2010Article
- Solitary pulmonary plasmacytoma: report of two cases.Medical oncology (Northwood, London, England) · 2010Article
Corrections and comments
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Authors and funding
10 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 kappa 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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.