全身性アミロイドーシスを伴ったCastleman病の1例

Abstract

A case of Castleman's disease accompanied by systemic amyloidosis is reported. A 77-year-old man with cardiac arrhythmia was admitted to our hospital. The cardiac arrhythmia improved after treatment with verapamil hydrochloride, but general fatigue persisted. Laboratory data showed severe macrocytic anemia, polyclonal hyper- gammopathy and accelerated erythrocyte sedimentation rate. Computed tomography of the abdomen revealed abnormal swelling of the mesenteric lymph node. In addition, this patient's condition was complicated with hypothyroidism and nephrotic syndrome. Although he underwent hemodialysis, he died of interstitial pneumonia which developed as a result of progressive renal failure. At autopsy, Castleman's lymphomas (plasma-cell type) and systemic amyloidosis were confirmed. Amyloid deposition was observed in the mesenteric mass, kidney, spleen, liver, lung, heart, and thyroid gland. Immunochemical study disclosed that the amyloid protein was immunoglobulin light chain, indicating immunocyte-derived (AL) amyloidosis. AL amyloidosis usually arises out of plasma cell dyscrasia (namely abnormal monoclonal proliferation of the plasma cells, such as multiple myeloma and plasmacytbma). Therefore this case suggests that the latent nature of malignant transformation is inherent in Cast- leman's disease though this disease is generally recognized as a benign reactive lympho- proliferative disease.