Idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia accompanied with cutaneous involvement and renal dysfunction.

Abstract

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.

• Authors: Saiko Kurosawa, Nobu Akiyama, Akira Ohwada, Masahiro Warabi, Matsuhiko Suenaga, Masaru Kojima, Junji Tomiyama
• Journal: Japanese journal of clinical oncology (Jpn J Clin Oncol) Vol. 39 Issue 10 Pg. 682-5 (Oct 2009) ISSN: 1465-3621 [Electronic] England
• PMID: 19586961 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Interleukin-6

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Humans
• Hypergammaglobulinemia (drug therapy, etiology, pathology)
• Interleukin-6 (blood)
• Lymphatic Diseases (complications, drug therapy, pathology)
• Male
• Plasma Cells (pathology)
• Renal Insufficiency (etiology, pathology)
• Skin Diseases (etiology, pathology)
• Treatment Outcome