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

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.

• Authors: Dosuke Iwadate, Eiko Hasegawa, Junichi Hoshino, Noriko Hayami, Keiichi Sumida, Masayuki Yamanouchi, Akinari Sekine, Masahiro Kawada, Rikako Hiramatsu, Tatsuya Suwabe, Naoki Sawa, Mitsuhiro Yuasa, Atsushi Wake, Takeshi Fujii, Kenichi Ohashi, Kenmei Takaichi, Yoshifumi Ubara
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 57 Issue 5 Pg. 701-706 (Mar 01 2018) ISSN: 1349-7235 [Electronic] Japan
• PMID: 29093408 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents, Alkylating
• Etoposide
• Doxorubicin
• Bence Jones Protein
• Melphalan

Topics

• Antineoplastic Agents, Alkylating (administration & dosage)
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Bence Jones Protein
• Biopsy
• Combined Modality Therapy
• Doxorubicin (therapeutic use)
• Etoposide (therapeutic use)
• Factor X Deficiency (complications, diagnosis, therapy)
• Hematopoietic Stem Cell Transplantation
• Humans
• Immunoglobulin Light-chain Amyloidosis (complications, diagnosis, therapy)
• Male
• Melphalan (administration & dosage)
• Middle Aged
• Remission Induction
• Transplantation, Autologous