Katoh Nagaaki, Matsushima Akira, Kurozumi Masahiro, Matsuda Masayuki, Ikeda Shu-ichi
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan.
Intern Med. 2014;53(17):1991-5. doi: 10.2169/internalmedicine.53.2065. Epub 2014 Sep 1.
A 52-year-old woman with a high serum alkaline phosphatase (ALP) level underwent a liver biopsy, which showed diffuse heavy deposition of Aκ amyloid, and was diagnosed as having immunoglobulin light chain (AL) amyloidosis. Although she received high-dose melphalan with stem cell transplantation and achieved a hematologic complete response (CR), her ALP level began to increase one year after treatment. Further examinations revealed that she was still in a CR state with dominant bone-type ALP, and re-biopsied liver specimens demonstrated marked regression of amyliod deposition, providing important evidence that the turnover of hepatic amyloid proteins can actually occur more rapidly than previously thought.
一名血清碱性磷酸酶（ALP）水平较高的52岁女性接受了肝脏活检，结果显示有弥漫性重度κ型淀粉样蛋白沉积，被诊断为免疫球蛋白轻链（AL）淀粉样变性。尽管她接受了大剂量美法仑联合干细胞移植并获得了血液学完全缓解（CR），但治疗一年后其ALP水平开始升高。进一步检查发现她仍处于以骨型ALP为主的CR状态，再次活检的肝脏标本显示淀粉样蛋白沉积明显消退，这提供了重要证据，表明肝脏淀粉样蛋白的更新实际上可能比之前认为的更快。
