The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency

A 55-year-old man was admitted to our institute to undergo evaluation for proteinuria (5.4 g/day) with lambda-type Bence-Jones protein (BJP). Primary amyloid light chain (AL) amyloidosis and acquired factor X deficiency were diagnosed. High-dose melphalan combined with autologous stem cell transplan...

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Detalles Bibliográficos
Autores principales: Iwadate, Dosuke, Hasegawa, Eiko, Hoshino, Junichi, Hayami, Noriko, Sumida, Keiichi, Yamanouchi, Masayuki, Sekine, Akinari, Kawada, Masahiro, Hiramatsu, Rikako, Suwabe, Tatsuya, Sawa, Naoki, Yuasa, Mitsuhiro, Wake, Atsushi, Fujii, Takeshi, Ohashi, Kenichi, Takaichi, Kenmei, Ubara, Yoshifumi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2017
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5874343/
https://www.ncbi.nlm.nih.gov/pubmed/29093408
http://dx.doi.org/10.2169/internalmedicine.9263-17
Descripción
Sumario:A 55-year-old man was admitted to our institute to undergo evaluation for proteinuria (5.4 g/day) with lambda-type Bence-Jones protein (BJP). Primary amyloid light chain (AL) amyloidosis and acquired factor X deficiency were diagnosed. High-dose melphalan combined with autologous stem cell transplantation was performed. After three years, the patient's proteinuria normalized, he was negative for urinary BJP, and his factor X activity improved to 105%. Serial renal biopsy showed no progression of amyloid deposition at a biopsy after 5 years, but showed a slight increase in the amyloid deposition after 11 years. This therapy can improve the prognosis of AL amyloidosis; however, there are limitations to the strategy.

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