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 non-Hodgkin'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 diffuse 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 differential diagnosis of localised pulmonary amyloidosis.
Key Words: marginal zone lymphoma • non-Hodgkin's lymphoma • amyloidosis
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Figure 1 Chest x ray demonstrating lung nodules in one patient. (A) Posteroanterior view of nodules; (B) lateral view of nodules.
Figure 2 Extranodal marginal zone lymphoma involving the lung. Note the lymphocytic infiltrate effacing the pulmonary architecture and the spread of the abnormal lymphocytes along the bronchovascular septa (haematoxylin and eosin stained; orignal magnification, x12).
Figure 3 Extranodal marginal zone lymphoma involving the lung. The neoplasm is composed of centrocyte-like lymphocytes that infiltrate the bronchiolar epithelium and form lymphoepithelial complexes (arrowheads). An atrophic germinal centre is also present (arrow) (haematoxylin and eosin stained; original magnification, x600).
Figure 4 Pronounced amyloid deposition in a pulmonary extranodal marginal zone lymphoma. The amorphous material on the right side of the photograph is amyloid (haematoxylin and eosin stained; original magnification, x600).