title: Rapid improvement of hyperammonemic encephalopathy by bortezomib treatment in IgD-type multiple myeloma.
abstract: A 74-year-old man visited our hospital with complaints of anorexia, weight loss, and impaired activities of daily living. He presented mild consciousness disturbance at the first visit, but specific causes were identified. The IgD level was>2,000 mg/dl and bone marrow biopsy was performed after aspiration failed due to excessive density. He was diagnosed with IgD/λ multiple myeloma (MM). He lapsed into a coma with an extremely high ammonia level of 484 μg/dl on day 8 after admission. His diagnosis was established as hyperammonemic encephalopathy (HE). He was treated with dexamethasone (Dex) pulse therapy and continuous hemodiafiltration. Minor improvement of hyperammonemia was achieved. Combination therapy with bortezomib and Dex was commenced. His ammonia level rapidly decreased and his mental state improved. HE accompanied by MM is rare and further studies are needed to clarify outcomes in response to treatment using the novel agent Bor. Although HE is potentially fatal, we found Bor to be rapidly effective against HE.
authors: Kansai Medical University, Hematology and Oncology.
authors: Maiko
authors: M
authors: Esaki
authors: Kazuyoshi
authors: K
authors: Ishii
authors: Yoshiko
authors: Y
authors: Azuma
authors: Yukie
authors: Y
authors: Tsubokura
authors: Hideaki
authors: H
authors: Yoshimura
authors: Masaaki
authors: M
authors: Hotta
authors: Takahisa
authors: T
authors: Nakanishi
authors: Shinya
authors: S
authors: Fujita
authors: Aya
authors: A
authors: Nakaya
authors: Atsushi
authors: A
authors: Satake
authors: Tomoki
authors: T
authors: Ito
authors: Shosaku
authors: S
authors: Nomura
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000069286:Bortezomib / Q000627:therapeutic use*; D001927:Brain Diseases / Q000209:etiology*; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology*; D007072:Immunoglobulin D / Q000276:immunology; D008297:Male; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy* / Q000276:immunology
chemical_list: D000970:Antineoplastic Agents; D007072:Immunoglobulin D; D000069286:Bortezomib