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We report on a patient who, at 31 years of age, was found tosuffer from sinus histiocytosis with massive lymphadenopathy (SHML; Rosai-Dorfman disease) with nodal and extranodal involvement as described previously. Five years later the patient presented with nephrotic syndrome caused by a generalized AA amyloidosis, and he subsequently died from pulmonary thromboembolism owing to renal vein thrombosis. Retrospective analysis of serum levels of C-reactive protein (CRP) showed that during the last 3 years before his death, he had a persistently elevated CRP level ranging from 73 to 161 mg/L, despite anti-inflammatory treatment with prednisolone, methotrexate, or 6-mercaptopurine. These figures indicate that the patient was probably suffering from a permanent acute phase response which, in the absence of any other evidence of a chronic inflammatory disease which commonly causes AA amyloidosis, was most likely owing to SHAM.

We report on a female patient, who, at the age of 63 years, was found to suffer from low-grade MALT-lymphoma localized at her right upper eyelid. At the time of initial diagnosis, clinical staging showed no further organ involvement. Within the following two months, nodular infiltrates occurred in both lungs. Histopathological investigation of the pulmonary lesions showed pulmonary involvement by the low-grade MALT-lymphoma associated with large globular amyloid deposits of lambda-light chain origin. Since the tumor cells of the MALT-lymphoma showed restriction to lambda-light chain the amyloid deposits in this case were interpreted as being related to the MALT-lymphoma.