PMID- 26502747 OWN - NLM STAT- MEDLINE DCOM- 20170328 LR - 20170328 IS - 1472-4146 (Electronic) IS - 0021-9746 (Linking) VI - 69 IP - 6 DP - 2016 Jun TI - Nodular pulmonary light chain deposition disease: an entity associated with Sjogren syndrome or marginal zone lymphoma. PG - 490-6 LID - 10.1136/jclinpath-2015-203342 [doi] AB - BACKGROUND: Light chain deposition disease (LCDD) is usually a systemic disorder characterised by non-amyloid monoclonal immunoglobulin light chain deposition in tissues. Localised nodular pulmonary (NP) LCDD is a rare and poorly characterised entity and, owing to the difficulties in diagnosis, limited data are available. METHODS: We investigated the clinical, radiological and pathological characteristics of a series of six confidently diagnosed cases of NPLCDD. RESULTS: There were three men and three women with ages ranging from 33 to 74 years. In all cases there were single or multiple pulmonary nodules, in one case associated with cysts. Two patients had no previous history of a lymphoproliferative or autoimmune disorder, two had Sjogren syndrome (SS) and two had extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Lung biopsies led to diagnoses of MALT lymphoma in four patients, including both of those with a previous history of lymphoma and one with SS. In five cases the diagnosis was confirmed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) and in one by electron microscopy. There was no evidence of systemic LCDD in any of the cases. Five patients had an indolent course in spite of limited therapeutic intervention while, in the patient who died, the cause of death was related to the spread of the lymphoma and was not due to the pulmonary lesions. CONCLUSIONS: NPLCDD is an indolent disease, in most cases associated with MALT lymphoma or autoimmune disease. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/ FAU - Arrossi, A V AU - Arrossi AV AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Merzianu, M AU - Merzianu M AD - Departement of Pathology, Roswell Park Cancer Institute, Buffalo, New York, USA. FAU - Farver, C AU - Farver C AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Yuan, C AU - Yuan C AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Wang, S H AU - Wang SH AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Nakashima, M O AU - Nakashima MO AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Cotta, C V AU - Cotta CV AD - R.J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20151026 PL - England TA - J Clin Pathol JT - Journal of clinical pathology JID - 0376601 RN - 0 (Immunoglobulin Light Chains) RN - Familial primary gastric lymphoma SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Immunoglobulin Light Chains/*metabolism MH - Lung/*pathology MH - Lymphoma, B-Cell, Marginal Zone/diagnostic imaging/*genetics MH - Lymphoma, Non-Hodgkin/diagnostic imaging/*genetics MH - Male MH - Middle Aged MH - Paraproteinemias MH - Sjogren's Syndrome/diagnostic imaging/*genetics MH - Stomach Neoplasms/diagnostic imaging/*genetics MH - Tandem Mass Spectrometry OTO - NOTNLM OT - IMMUNOGLOBULIN OT - LYMPHOMA OT - PULMONARY PATHOLOGY EDAT- 2015/10/28 06:00 MHDA- 2017/03/30 06:00 CRDT- 2015/10/28 06:00 PHST- 2015/08/17 00:00 [received] PHST- 2015/10/03 00:00 [accepted] PHST- 2015/10/28 06:00 [entrez] PHST- 2015/10/28 06:00 [pubmed] PHST- 2017/03/30 06:00 [medline] AID - jclinpath-2015-203342 [pii] AID - 10.1136/jclinpath-2015-203342 [doi] PST - ppublish SO - J Clin Pathol. 2016 Jun;69(6):490-6. doi: 10.1136/jclinpath-2015-203342. Epub 2015 Oct 26.