The Long-Term Outcomes After VAD Plus SCT Therapy in a Patient With AL Amyloidosis and Severe Factor X Deficiency | oneAMYLOIDOSISvoice
Scientific Articles

The Long-Term Outcomes After VAD Plus SCT Therapy in a Patient With AL Amyloidosis and Severe Factor X Deficiency

key information

source: Internal Medicine

year: 2018

authors: Iwadate D, Hasegawa E, Hoshino J, Hayami N, Sumida K, Yamanouchi M, Sekine A, Kawada M, Hiramatsu R, Suwabe T, Sawa N, Yuasa M, Wake A, Fujii T, Ohashi K, Takaichi K, Ubara Y

summary/abstract:

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.
 
organization: Toranomon Hospital, Japan; Graduate School of Medicine, Japan

DOI: 10.2169/internalmedicine.9263-17

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