Primary Sjögren's syndrome associated with hyaline-vascular type of Castleman's disease and autoimmune idiopathic thrombocytopenia.

We describe a case of primary Sjögren's syndrome complicated by hyaline-vascular type of Castleman's disease and autoimmune idiopathic thrombocytopenia. This type of Castleman's disease was diagnosed by biopsy of a right axillary lymph node 7 years after the onset of Sjögren's syndrome. The specimen showed small hyaline-vascular lymphoid follicles and interfollicular capillary proliferation. Serum IL-6 was slightly increased, but systemic manifestations, such as fever or weight loss, were not present. Hyaline-vascular type of Castleman's disease should be considered a lymphoproliferative disorder associated with Sjögren's syndrome.