Blood stem cell transplantation to treat cystic lung light chain deposition disease

Borgne, A. Le; Prévôt, Grégoire; Rouquette, Isabelle; Huynh, Anne; Têtu, L.; Projetti, Fabrice; Carreiro, M.; Borie, Raphaël; Jaccard, Arnaud; Récher, Christian; Didier, A.

doi:10.1183/13993003.00577-2014

Cited by 8 publications

(9 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…12 According to the characteristic clinical, radiologic, and molecular analyses, Colombat et al 11,12 claimed that CL-LCDD is a separate clinicopathologic entity of localized lung disease. Another case was reported by Borgne et al 7 in 2015, which was secondary to lymphoplasmacytic lymphoma and treated successfully with chemotherapy and autologous peripheral blood stem cell transplantation (autoPBSCT).…”

mentioning

confidence: 99%

“…3 To date, there have been 46 reported cases of pulmonary light chain deposition disease (PLCDD). 6,7 The pulmonary involvement of light chain deposits has been mainly reported as part of a systemic disorder, whereas in a few cases the disease was limited to the lungs. 6 Some clinical features of LCDD associated with the respiratory organs have been identified, including nodules, cysts, airway involvement, 8 and bronchiectasis.…”

mentioning

confidence: 99%

“…6 Recently, cases of cystic lung LCDD (CL-LCDD), which is a unique form of diffuse PLCDD that shows innumerable lung cysts, were reported. 7,10,11 Seven patients showed rapid progression of respiratory failure and underwent lung transplantation. 10,11 In some of these cases, there was no proliferation of plasma cells in bone marrow, whereas the specific clonal expansion of B cells with an unmutated IGHV4-34/IGKV1 receptor was found in the explanted lung samples.…”

mentioning

confidence: 99%

See 2 more Smart Citations

A 56-Year-Old Woman With Multiple Pulmonary Cysts and Severe Chest Pain

Kato

Muto

Hishima

et al. 2018

Chest

View full text Add to dashboard Cite

A 56-year-old woman presented to our hospital with a 4-month history of worsening chest pain. She denied having any respiratory symptoms, such as dyspnea, sputum, cough, or hemoptysis, or any history of smoking or exposure to dusts. One year previously she had a vertebral fracture. There was no specific family history, including pulmonary or autoimmune diseases. Chest CT performed 3 years earlier showed multiple thin-walled pulmonary cysts, although no further investigations were performed.

show abstract

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

A 56-Year-Old Woman With Multiple Pulmonary Cysts and Severe Chest Pain

Kato

Muto

Hishima

et al. 2018

Chest

View full text Add to dashboard Cite

show abstract

“…Pulmonary LCDD is an indolent or slowly progressive disease [203]. No recurrence after a 4-year follow-up was observed after lung transplantation [201,207]. Unlike localized disease, treatment of systemic LCDD aims at reducing the serum free light chain level to limit lung deposits.…”

Section: Pulmonary Light-chain Deposition Diseasementioning

confidence: 99%

“…Optimal treatment remains to be defined. Chemotherapy can be associated with peripheral blood stem cell transplantation [207]. …”

Section: Pulmonary Light-chain Deposition Diseasementioning

confidence: 99%

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

View full text Add to dashboard Cite

In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

show abstract