Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma.
J Clin Pathol
; 69(6): 490-6, 2016 Jun.
Article
em En
| MEDLINE
| ID: mdl-26502747
ABSTRACT
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 74â 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 Sjögren 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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Gástricas
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Linfoma não Hodgkin
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Síndrome de Sjogren
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Cadeias Leves de Imunoglobulina
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Linfoma de Zona Marginal Tipo Células B
/
Pulmão
Tipo de estudo:
Risk_factors_studies
Limite:
Adult
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Aged
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Female
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Humans
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Male
/
Middle aged
Idioma:
En
Revista:
J Clin Pathol
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Estados Unidos