Pulmonary marginal zone lymphoma of MALT type as a cause of localised pulmonary amyloidosis

Abstract

Aim-To describe six patients with pulmonary marginal zone lymphoma in whom amyloid deposition was identified. Marginal zone lymphoma is a recently recognised type of low grade nonHodgkin's lymphoma. Methods-A computerised search was performed of all patients seen at the Mayo Clinic with a diagnosis of pulmonary amyloidosis. Six patients with pulmonary amyloidosis who had biopsy confirmed extranodal marginal zone lymphoma of mucosa associated lymphoid tissue type were identified. All were women, ranging in age from 45 to 85 years. Results-Five patients had amyloid deposition in conjunction with pulmonary marginal zone lymphoma at the time of the original diagnosis. One patient was referred for evaluation of localised pulmonary amyloidosis and was found to have coexisting pulmonary marginal zone lymphoma. Clinical presentation was limited to pulmonary symptoms (two of the six) or constitutional symptoms (two), or was asymptomatic (two). In all six cases, initial findings of nodular densities on screening chest roentgenograms led to further evaluation and eventual lobectomy; these findings included multiple pulmonary nodules (four), single nodule (one), and single nodule with diVuse bilateral interstitial infiltrates (one). Bone marrow was examined in five patients and was normal in all. Protein studies performed in four patients revealed no monoclonal protein.No patients had manifestations of systemic amyloidosis, such as renal, neurological, or cardiac involvement, at a median follow up of 50 months. Four of the six patients remain alive at a median of five years. Conclusions-Pulmonary marginal zone lymphoma may be found in association with localised amyloid deposition and should be considered in the diVerential diagnosis of localised pulmonary amyloidosis. (J Clin Pathol 2001;54:642-646)

The lung in amyloidosis

“…In these patients, the relative paucity of presenting symptoms and physical examination findings, and the absence of systemic amyloidosis after a rigorous clinical evaluation corroborated the localised nature of the amyloid in the pulmonary marginal zone lymphoma. The underlying lymphoproliferative disorder might be subtle, but sensitive methods reveal a clonal B-cell population in most cases [16,31,32]. The finding of monotypic lymphoid cells on immunohistochemical analysis confirms the diagnosis of lymphoma.…”

“…One study, from the Mayo Clinic [31], reported six cases in which this association could be made without the coexistent systemic amyloidosis. In these patients, the relative paucity of presenting symptoms and physical examination findings, and the absence of systemic amyloidosis after a rigorous clinical evaluation corroborated the localised nature of the amyloid in the pulmonary marginal zone lymphoma.…”

Unusual cystic presentation of pulmonary nodular amyloidosis associated with MALT-type lymphoma

“…As the authors' first diagnosis based on fine-needle biopsy was restricted to pulmonary nodular amyloidosis, it must be kept in mind that the amyloidosis may be isolated or may accompany either benign or malignant lymphoproliferations, including nodular lymphoid hyperplasia, lymphoid interstitial pneumonia and lymphoma, in addition to connective tissue diseases and HIV infection [1,4]. Other malignant lung tumours can also exhibit amyloid deposits, such as carcinoids, which may contain an amyloid stroma, which is likely to be composed of calcitonin-related gene peptide or to be neuropeptide derived [15], or squamous cell and bronchioloalveolar carcinomas, where amyloid deposits are more related to systemic amyloidosis [16,17].…”

“…A ssociation of pulmonary amyloidosis with lymphoma is a classical albeit rare finding [1][2][3][4], reported mainly with extranodal marginal zone B-cell lymphoma of mucosaassociated lymphoid tissue (MALT lymphoma) of gastrointestinal [5,6] or pulmonary origin [1,2]. Pulmonary nodular amyloidosis is a localised form of pulmonary amyloidosis, in addition to tracheobronchial and diffuse alveolo-septal amyloidosis, characterised histologically by extracellular deposits of amyloid-type AL protein, homologous to the variable portion of immunoglobulin light chains, predominantly of l type.…”

Amyloidosis of the Lung

“…44 In the past, nodular pulmonary amyloidosis and primary pulmonary lymphoma with amyloid production were thought to be 2 fundamentally different processes. 45,46 However, many experts now believe that most cases of nodular pulmonary amyloidosis are the result of an underlying lymphoproliferative disorder in the spectrum of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). 33 The underlying lymphoproliferative disorder might be subtle, but sensitive methods reveal a clonal B-cell population in most cases.…”