Facial flushing and localized Castleman's disease: an extension of the POEMS syndrome phenotype – reply

Abstract

Dear Sir, We thank Dr Ma et al. for their comments concerning our article [1]. Castleman's disease is known as angiofollicular lymph node hyperplasia, which is separated into localized type and multicentric type. Multicentric Castleman's disease (MCD) presents with systemic symptoms and multiple lymphadenopathy. Clinical findings in patients with MCD are fatigue, fever, loss of weight, night sweats, anorexia and oedema; laboratory findings of MCD are anaemia, elevated ESR, hypergammagloblinaemia, and hypoalbuminaemia [2]. Some of these findings are consistent with POEMS syndrome, and the lymph node of the patients with POEMS syndrome shows Castleman's disease in about 60%[3]. Therefore, POEMS syndrome might be associated with Castleman's disease. The causes of reactive amy loidosis inc1ude chronic inflammatory disorders (e.g. rheumatoid arthritis), chronic infections and malignancy [4]. Ordi J et al. reported and reviewed amyloidosis associated with Castleman's disease; six of eight patients with amyloidosis are reactive amyloidosis [5]. Castleman's disease is classified histologically as hyaline-vascular type, plasma cell type and mixed type. In the hyaline-vascular type, the germinal centres are surrounded by multiple concentric layers of lymphocytes, which are penetrated radial capillaries surrounded by hyaline material. In the plasma-cell type, the interfollicular areas are occupied by extensive sheets of plasma cells [6]. Although the plasma cells in hilar and mesenteric lymph nodes were observed [4], there was neither swelling in the nodes nor histopathological features consistent with Castleman's disease at autopsy in our case. Castleman's disease might not be responsible for the reactive amyloidosis in our case. Received 7 November 2002; accepted 11 November 2002. Dr Y. Kihara, Third Department of Internal Medicine, University of Occupational and Environmental Health, Japan, School of Medicine, Kitakyushu, Japan. (fax: +81 93 692 0107: e-mail: [email protected]).