Histological proof remains the gold standard for the diagnosis of amyloidosis. Nuclear medicine imaging techniques are able to determine the amyloid load in the body. Currently, the best imaging modality is I-123-SAP scintigraphy. This modality has high sensitivity for detecting amyloid deposits in all amyloid subtypes. Involvement of liver and spleen can be visualized before clinical signs are present. The addition of single photon emission computed tomography improves the differentiation of overlying organs. However, I-123-SAP is not FDA approved. Its availability is limited to two centres in Europe....

PURPOSE: Cardiac amyloidosis is a rare disorder, but it may lead to potentially life-threatening restrictive cardiomyopathy. Cardiac manifestations frequently occur in primary amyloidosis (AL) and familial amyloidosis (ATTR), but are uncommon in secondary amyloidosis (AA). Echocardiography is the method of choice for assessing cardiac amyloidosis. Amyloid deposits impair the function of sympathetic nerve endings. Disturbance of myocardial sympathetic innervations may play an important role in the remodelling process. (123)I-MIBG can detect these innervation changes. METHODS: Patients with biopsy-proven amyloidosis underwent general work-up, echocardiography and (123)I-MIBG scintigraphy. Left ventricular...

BACKGROUND:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease (RDD)) is a rare entity with pathological accumulation of specific, activated macrophages, mainly in cervical lymph nodes. However, in half of patients its primary presentation is extranodal. Its aetiology is unclear; several clues point in an immunological direction. Common clinical presentation is massive cervical lymphadenopathy, but nearly half of all patients suffer from extranodal manifestation at very diverse localizations. Diagnosis is based on histopathological findings: emperipolesis and S-100 positive histiocytes. Taking into account its benign character, caution in implementing therapy is...