Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. We report a fatal case of ECD with extensive cardiac involvement associated with a marginal zone lymphoma and monoclonal proteinemia in a young man. This is the first reported association of ECD with a monoclonal gammopathy or a lymphoma.

Extranodal marginal zone lymphoma of MALT, previously known as MALT lymphoma, is a low grade B-cell Non-Hodgkin's lymphoma (NHL). Its most frequent locations are the gastrointestinal tract and the lungs while that of the colon is rare. Involvement of multiple mucosal sites is not a frequent finding but it does occur. We describe a case of a 70-year-old man who presented with extranodal marginal...

Extra-nodal MZBCL of MALT type (7% of all NHL) is an indolent disease involving most often the stomach, where it usually follows chronic gastritis due to Helicobacter pylori (HP) infection. The disease may also localize in the lung, the thyroid, the salivary gland, the orbit. Splenic MZBCL (1% of all NHL on histologic samples) usually, though not invariably, presents splenomegaly associated cir...

Chronic HCV infection can predispose to different lymphoproliferative disorders including lymphomas (diffuse large B-cell lymphoma, marginal zone lymphoma, lymphoplasmacytic lymphoma) and plasma cell disorders (monocolonalgammopathies) [1,2]. Kelaidi et al reported a case of large granular lymphocyte leukemia occurring in association with Marginal Zone Lymphoma and HCV and responding to interfe...

BACKGROUND Primary cutaneous B-cell lymphoma manifested by anetoderma has been reported in 7 cases. In all, the secondary anetoderma developed in lesions of marginal-zone lymphoma or posttransplant lymphoproliferative disorder resembling marginal-zone lymphoma. The mechanisms underlying the destruction of elastic tissue in anetoderma are unclear. However, there is growing evidence linking prima...

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 ext...