ボルテゾミブ・シクロホスファミド・デキサメタゾン(VCD) 3剤併用療法にて完全寛解を達成した血清KL-6高値多発性骨髄腫  [in Japanese] Complete remission achieved by a combination regimen with bortezomib, cyclophosphamide, and dexamethasone in a multiple myeloma patient with elevated serum KL-6 level  [in Japanese]

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Author(s)

    • 奥野 豊 OKUNO Yutaka
    • 熊本大学大学院生命科学研究部 血液内科 Department of Hematology, Kumamoto University School of Medicine
    • 西村 直 NISHIMURA Nao
    • 熊本大学大学院生命科学研究部 血液内科 Department of Hematology, Kumamoto University School of Medicine
    • 畑 裕之 HATA Hiroyuki
    • 熊本大学大学院生命科学研究部 血液内科 Department of Hematology, Kumamoto University School of Medicine

Abstract

79歳女性。約4年間MGUSとして外来にて経過観察されていたが,複視が出現してきたため,多発性骨髄腫への移行とそれに伴う形質細胞腫を疑われ当院入院となる。髄外性病変として頭蓋内3ヶ所と腰椎に形質細胞腫を認め,眼窩内の腫瘤が複視の原因と考えられた。KL-6は1,409 U/<i>l</i>と高値を示したが,CTでは肺野に間質影などの病変を認めなかった。KL-6高値多発性骨髄腫の報告例が予後不良であったため,高齢ではあったがbortezomib, cyclophosphamide, dexamethasoneによる3剤併用(VCD)療法を行ったところ,6サイクル終了時点でstringent CRを達成し,それと同時に複視は軽快し,またKL-6も正常化した。従って,髄外腫瘤を伴うKL-6高値多発性骨髄腫である本症例に対してVCD療法は有効な治療法であり,その予後を改善した。

A 79-year-old woman suffering from double vision after a 4-year history of MGUS was referred to our hospital. MRI revealed that she had three intracranial plasmacytoma masses and one spinal plasmacytoma mass. Bone marrow aspirates showed 52.4% plasma cell infiltration and immunoelectrophoresis identified serum IgG-M protein, leading to a diagnosis of IgG-type multiple myeloma. IgG was elevated to 3,355 mg/d<i>l</i> and urine type Bence-Jones protein was positive. KL-6, a membrane-bound glycoprotein encoded by Mucin 1 and a marker of interstitial pneumonia, was also elevated to 1,409 mg/d<i>l</i>, but computed tomography of the lungs revealed no obvious pulmonary lesions. Previously reported studies showing that myeloma patients with elevated KL-6 might have a poor prognosis prompted us to treat this patient with a three-drug (bortezomib, cyclophosphamide, and dexamethasone: VCD) combination regimen. When 6 cycles of the regimen had been completed, no M-protein was detectable in her serum. Furthermore, κ free light chain had significantly decreased from 12,700 to 24.8 mg/<i>l</i>. In addition, <sup>18</sup>F-FDG PET/CT revealed reduced mass sizes and no <sup>18</sup>F-FDG uptakes by plasmacytomas. Thus, she was defined as having achieved a stringent complete remission (sCR). We therefore concluded that the VCD combination regimen was highly effective in this multiple myeloma patient with KL-6 elevation.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 55(4), 461-465, 2014

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130004920674
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    025488718
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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