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

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

doi:10.2169/internalmedicine.9263-17

CASE REPORTS

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

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

Author information

Dosuke Iwadate
Nephrology Center, Toranomon Hospital, Japan
Eiko Hasegawa
Nephrology Center, Toranomon Hospital, Japan
Junichi Hoshino
Nephrology Center, Toranomon Hospital, Japan
Noriko Hayami
Nephrology Center, Toranomon Hospital, Japan
Keiichi Sumida
Nephrology Center, Toranomon Hospital, Japan
Masayuki Yamanouchi
Nephrology Center, Toranomon Hospital, Japan
Akinari Sekine
Nephrology Center, Toranomon Hospital, Japan
Masahiro Kawada
Nephrology Center, Toranomon Hospital, Japan
Rikako Hiramatsu
Nephrology Center, Toranomon Hospital, Japan
Tatsuya Suwabe
Nephrology Center, Toranomon Hospital, Japan
Naoki Sawa
Nephrology Center, Toranomon Hospital, Japan
Mitsuhiro Yuasa
Department of Hematology, Toranomon Hospital, Japan
Atsushi Wake
Department of Hematology, Toranomon Hospital, Japan
Takeshi Fujii
Department of Pathology, Toranomon Hospital, Japan
Kenichi Ohashi
Department of Pathology, Toranomon Hospital, Japan
Department of Pathology, Yokohama City University, Graduate School of Medicine, Japan
Kenmei Takaichi
Nephrology Center, Toranomon Hospital, Japan
Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan
Yoshifumi Ubara
Nephrology Center, Toranomon Hospital, Japan
Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan

Keywords: AL amyloidosis, factor X deficiency, vincristine, adriamycin, and dexamethasone (VAD), high-dose melphalan, autologous stem cell transplantation (SCT)

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 5 Pages 701-706

DOI https://doi.org/10.2169/internalmedicine.9263-17

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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.

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