Systemic Amyloidosis Associated with Giant Lymph Node Hyperplasia

Abstract

This report describes 2 cases of systemic amyloidosis associated with giant lymph node hyperplasia (GLNH). Case 1: Multicentric GLNH, plasma cell (PC) type, in a 39-year-old man was accompanied by systemic manifestations, such as fever, anemia, generalized peripheral lymphadenopathy, and polyclonal hypergammaglobulinemia. Oner year later, nephrotic syndrome occurred, and then hypothyroid goiter followed. Thyroid tissue was almost completely replaced with eosinophilic material. The material stained with Congo red and anti-AA. Case 2: Monocentric GLNH, PC type, in a 53-year-old man was accompanied by anemia and fever of 3 years’ duration. No peripheral lymphadenopathy was noted. Intermittent Bence Jones proteinuria (λ type) was found. Abdominal CT scan disclosed a mesenteric mass and hepatosplenomegaly. Biopsy specimens from the mass, liver and small intestine showed GLNH, PC type, with AL-amyloid and λ light chain deposits. Immunofluorescent study showed that the majority of plasma cells contained cytoplasmic λ light chain. To our knowledge, there are no previous reports concerning AL-amyloidosis developing in patients with GLNH. The type of associated amyloidosis in GLNH seems to depend on whether the plasma cells are polyclonal or monoclonal. Here, we would like to add our comments on the biologic behavior of GLNH.