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...

Descripción completa

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
_version_ 1783310147266805760
author 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
author_facet 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
author_sort Iwadate, Dosuke
collection PubMed
description 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.
format Online
Article
Text
id pubmed-5874343
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher The Japanese Society of Internal Medicine
record_format MEDLINE/PubMed
spelling pubmed-58743432018-03-29 The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency 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 Intern Med Case Report 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. The Japanese Society of Internal Medicine 2017-11-01 2018-03-01 /pmc/articles/PMC5874343/ /pubmed/29093408 http://dx.doi.org/10.2169/internalmedicine.9263-17 Text en Copyright © 2018 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
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
The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title_full The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title_fullStr The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title_full_unstemmed The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title_short The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency
title_sort long-term outcomes after vad plus sct therapy in a patient with al amyloidosis and severe factor x deficiency
topic Case Report
url 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
work_keys_str_mv AT iwadatedosuke thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hasegawaeiko thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hoshinojunichi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hayaminoriko thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sumidakeiichi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT yamanouchimasayuki thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sekineakinari thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT kawadamasahiro thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hiramatsurikako thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT suwabetatsuya thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sawanaoki thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT yuasamitsuhiro thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT wakeatsushi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT fujiitakeshi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT ohashikenichi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT takaichikenmei thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT ubarayoshifumi thelongtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT iwadatedosuke longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hasegawaeiko longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hoshinojunichi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hayaminoriko longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sumidakeiichi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT yamanouchimasayuki longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sekineakinari longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT kawadamasahiro longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT hiramatsurikako longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT suwabetatsuya longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT sawanaoki longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT yuasamitsuhiro longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT wakeatsushi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT fujiitakeshi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT ohashikenichi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT takaichikenmei longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency
AT ubarayoshifumi longtermoutcomesaftervadplusscttherapyinapatientwithalamyloidosisandseverefactorxdeficiency

Ejemplares similares