Ueda Hiroyuki, Kuno Hideaki, Takahashi Daisuke, Katsuma Ai, Kimura Ai, Nakashima Akio, Kato Junichiro, Momoki Mamiko, Ohba Rie, Dobashi Nobuaki, Yamamoto Izumi, Kawamura Tetsuya, Miyazaki Yoichi, Yokoo Takashi
Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Nishi-shinbashi 3-25-8, Minato-ku, Tokyo, 105-8461, Japan.
Division of Hematology and Oncology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.
CEN Case Rep. 2020 May;9(2):165-172. doi: 10.1007/s13730-020-00448-y. Epub 2020 Jan 23.
The immunoglobulin (Ig) D type is a rare variant of multiple myeloma (MM), that accounts for 1-2% of all cases. Compared to the more common types of MM, IgD MM is known to have more severe symptoms at presentation, and a poorer prognosis. A woman was admitted to our hospital for severe acute kidney disease and disorder (AKD) and back pain, and was started on hemodialysis. The renal biopsy revealed light chain cast nephropathy. She was diagnosed with IgD-λ MM based on Bence-Jones protein expression and high IgD serum levels, and started bortezomib therapy with plasma exchange (PE). After three sessions of PE, the serum free light chain levels decreased by 92%, and she was withdrawn from dialysis. The patient underwent autologous transplantation and is still in remission, demonstrating the benefits of a bortezomib-based regimen in combination with PE for IgD MM with AKD.
免疫球蛋白(Ig)D型是多发性骨髓瘤(MM)的一种罕见变异类型,占所有病例的1%-2%。与更常见的MM类型相比,IgD MM在发病时症状更严重,预后更差。一名女性因严重急性肾疾病和紊乱(AKD)及背痛入住我院,并开始进行血液透析。肾活检显示为轻链管型肾病。根据本斯-琼斯蛋白表达和高IgD血清水平,她被诊断为IgD-λ MM,并开始接受硼替佐米联合血浆置换(PE)治疗。经过3次PE治疗后,血清游离轻链水平下降了92%,她停止了透析。该患者接受了自体移植,目前仍处于缓解期,这表明基于硼替佐米的方案联合PE治疗伴有AKD的IgD MM具有益处。