Solitary plasmacytoma of the lung with light chain extracellular deposits: a case report and review of the literature

Light chain deposition disease (LCDD) infrequently affects the lungs and usually causes damage to the parenchyma, while bronchial involvement appears to be very rare. The present authors report the case of a 64-yr-old female with LCDD characterised by asymptomatic airway involvement.Ten months after excision of a poorly differentiated vaginal carcinoma, a routine chest computed tomography (CT) scan revealed two lung cysts, several bilateral nodules and diffuse bronchial thickening. Pulmonary function tests were normal. Fibreoptic bronchoscopy showed marked diffuse mucosal thickening with highly conspicuous vascular plexuses. Nonamyloidal deposits were found in the bronchial wall, but no definite diagnosis could be proposed.On follow-up, the patient was still asymptomatic and the CT scan and endoscopic appearance remained unchanged. The final diagnosis of k LCDD was established 18 months later by another series of bronchial biopsies with frozen samples. Interestingly, electron microscopy showed dense granular deposits associated with nonamyloidal fibrils. An increased number of lung cysts were observed 32 months after identification of bronchial abnormalities, confirming the progressive nature of the disease. No extrapulmonary deposits or immunoproliferative disorder were found.In conclusion, light chain deposition disease, which may remain latent for several years, can entirely involve large airways and may be diagnosed by bronchial biopsy.

Section: Discussionmentioning

confidence: 81%

“…The possibility of lung nodules and diffuse pulmonary interstitial disease has been reported, but, to date, only seven patients with pulmonary nodular-type LCDD have been reported in the literature [11][12][13][14][15]. These patients were mainly asymptomatic.…”

Section: Discussionmentioning

confidence: 99%

Light chain deposition disease involving the airways: diagnosis by fibreoptic bronchoscopy

Colombat¹,

Gounant²,

Mal³

et al. 2007

“…Like AL amyloidosis, NAMIDD predominantly involves the kidney and the heart, but lung involvement is exceptional, with only 17 cases reported [3][4][5][6][7][8][9][10][11][12][13]. Pulmonary NAMIDD may present as multiple parenchymal nodules (nine cases) [3][4][5][6][7][8][9] or as a single mass (three cases) [9,10].…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary NAMIDD may present as multiple parenchymal nodules (nine cases) [3][4][5][6][7][8][9] or as a single mass (three cases) [9,10]. A unique presentation of lymphangioleiomyomatosis-like polycystic lung disease has also been described recently (four cases) [11,12].…”

Section: Discussionmentioning

confidence: 99%

Respiratory failure with diffuse bronchiectases and cryoglobulinaemia

Girard¹,

Vasiljevic²,

Cottin³

et al. 2008

A 48-yr-old nonsmoking female presented with a 5-yr history of recurrent bronchitis with progressive exercise dyspnoea. She had been diagnosed with a small monoclonal gammopathy of undermined significance (MGUS) 6 yrs previously. Inspiratory vital capacity was 4.7 L (125% predicted) and forced expiratory volume in one second/forced vital capacity (FVC) ratio was 79%. Carbon monoxide transfer factor was markedly impaired (35% pred), and arterial oxygen tension (Pa,O 2 ) decreased from 9.2 to 6.0 kPa at exercise (40 W, 10 min). A thoracic computed tomography (CT) scan was performed ( fig. 1). Fibreoptic bronchoscopy found inflammation of the bronchial mucosa with candle stain-like deposits on the trachea and large bronchi, without any purulent secretion within the respiratory tract ( fig. 2). The histopathological aspect of bronchial biopsies is shown in figure 3. Blood tests disclosed type I cryoglobulinaemia composed of a 32 g?L -1 monoclonal immunoglobulin (Ig)Mk serum component (at 37uC). Blood cell count was normal. Bone marrow examination showed malignant lymphoplasmacytic cells representing 30% of total cells, with a monotypic surface expression of IgMk. The C-reactive protein level was 21 mg?L -1 . Extensive renal, cardiac and hepatic evaluations were normal. As severe hypoxaemia and reduction of carbon monoxide transfer factor were unexplained, the patient underwent videothoracoscopic lung biopsies.

“…Lung involvement is asymptomatic and usually diagnosed at the time of autopsy. Since 1987, various authors have reported 10 cases of nodular LCDD restricted to the lung [5][6][7][8][9][10]. They may be single or multiple nodules.…”

mentioning

confidence: 99%

Pathomechanisms of cyst formation in pulmonary light chain deposition disease

Colombat¹,

Caudroy²,

Lagonotte³

et al. 2008

Cystic lung light chain deposition disease (CL-LCDD) is a recently described rare disorder characterised by numerous cysts and diffuse monoclonal nonamyloid light chain deposits surrounded by macrophagic giant cells. The mechanisms responsible for cyst development remain unknown.The objectives of the present study were to analyse the major components of the pulmonary extracellular matrix in CL-LCDD and to determine the influence of metalloproteinases (MMPs) by comparison with other cystic lung disorders.A virtually complete degradation of the elastic network was found in CL-LCDD. To a lesser degree, loss of fibrillar and basement membrane collagens was also observed. Macrophagic giant cells expressed MMP-1, MMP-2, MMP-9, MMP-12 and MMP-14 and in situ zymography highlighted a strong gelatinolytic activity. As in CL-LCDD, cystic lesions in Langerhans' cell histiocytosis (LCH) and lymphangioleiomyomatosis (LAM) were characterised by the lack of elastic fibres. Similarly, MMP were expressed in CL-LCDD and LCH but the labelled cells were different. In contrast, few MMPs were detected in LAM.In conclusion, elastolysis is common to cystic lung light chain deposition disease and other cystic lung disorders, suggesting its implication in cyst formation. Moreover, in cystic lung light chain deposition disease, a role of metalloproteinases in elastolysis is strongly indicated by the metalloproteinase expression and activity pattern.