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Japanese Journal of Clinical Oncology Advance Access published online on July 8, 2009
Japanese Journal of Clinical Oncology, doi:10.1093/jjco/hyp073
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© The Author (2009). Published by Oxford University Press. All rights reserved

Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hypergammaglobulinemia Accompanied with Cutaneous Involvement and Renal Dysfunction

Saiko Kurosawa1,6, Nobu Akiyama2, Akira Ohwada1, Masahiro Warabi3, Matsuhiko Suenaga4, Masaru Kojima5 and Junji Tomiyama1

1 Department of Hematology, Tokyo Metropolitan Bokutoh General Hospital, Tokyo
2 Department of Internal Medicine, Teikyo University School of Medicine, Tokyo
3 Department of Pathology, Tokyo Metropolitan Bokutoh General Hospital
4 Department of Nephrology, Tokyo Metropolitan Bokutoh General Hospital, Tokyo
5 Department of Anatomic and Diagnostic Pathology, Dokkyo Medical University School of Medicine, Tochigi, Japan

For reprints and all correspondence: Saiko Kurosawa, Department of Hematology, Tokyo Metropolitan Bokutoh General Hospital, Tokyo, Japan. E-mail: skurosaw{at}ncc.go.jp

Received February 14, 2009; accepted June 2, 2009

Idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hypergammaglobulinemia has been proposed as a new disease entity resembling the plasma cell type of multicentric Castleman's disease. Here, we report a case of IPL accompanied by renal failure and skin involvement. A 35-year-old man was admitted for advanced renal failure, anemia, systemic lymphadenopathy and skin rashes. Laboratory examinations indicated polyclonal hypergammaglobulinemia and elevated serum interleukin-6 (IL-6). Biopsy of a cervical lymph node revealed follicular hyperplasia with normal germinal centers, sheets of polyclonal proliferating plasma cells and the absence of marked proliferation of blood vessels in the interfollicular area. Lesions of the kidney and skin also had pathological characteristics of IPL. Following a diagnosis of IPL, corticosteroid therapy successfully improved the anemia and hypergammaglobulinemia, and serum IL-6 levels decreased to a normal range. This case may give suggestions about diagnosing and preventing the progression of complications from this disease entity.

Key Words: IPL • renal failure • skin involvement • corticosteroid therapy

6 Present address: Department of Stem Cell Transplantation, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.


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