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

Internal Medicine
Dosuke IwadateYoshifumi Ubara

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.

References

Jan 21, 2012·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Yoshinari HattoriKenmei Takaichi
Sep 30, 2014·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Jean D SipePer Westermark
Oct 23, 2015·British Journal of Haematology·Efstathios Kastritis, Meletios A Dimopoulos
Jan 1, 2016·Lancet·Ashutosh D WechalekarPhilip N Hawkins

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Citations

Feb 20, 2021·Clinical and Experimental Nephrology·Yoshifumi UbaraUNKNOWN Committee of Practical Guide for Kidney Biopsy 2020

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Methods Mentioned

BETA
electrophoresis
biopsy

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