頸部腫脹で発見された原発性マクログロブリン血症を伴うアミロイドーシス例

Abstract

A 76-year-old man had been aware of the swelling of the posterior neck on both sides for a year. When he underwent health screening, an abnormal shadow in the mediastinum was found on CT imaging. The local findings at the first visit, revealed multiple swelling in the lateral and posterior neck with no tenderness. Lymphadenopathy from the mediastinum to the neck in the cervicothoracic was suspected from the CT findings. We removed a lymph node under general anesthesia, but the specimen lacked diagnostic characteristics. The lymph nodes of the submandibular gland were also removed. The specimen showed no structure of the lymph node but an eosinophilic lesion was observed. Congo red staining was performed. Based on the results of the general examination, a diagnosis of primary macroglobulinemia was made. Amyloidosis is a pathological condition in which amyloid with a fiber structure is deposited in the extracellular tissue of various organs. The present patient was diagnosed as having amyloidosis with underlying primary macroglobulinemia. Deposition of amyloid material is frequently found in the heart, tongue, skeletal muscle, and intestinal tract, but very rarely in the neck. The present case had no subjective symptoms other than cervical lymphadenopathy, and the diagnosis was made by biopsy. Reports of amyloidosis that was discovered through swelling of the neck are very scarce with only two cases in Japan, and five cases overseas.