Clinical benefits of bortezomib-containing regimens for newly diagnosed AL amyloidosis with severe cardiac impairment

TSUKUNE Yutaka, YAHATA Yuriko, SASAKI Makoto, HIKI Makoto, TSUTSUI Miyuki, HAMANO Yasuharu, ITOH Seigo, MIYAZAKI Tetsuro, DOHI Tomotaka, MARUYAMA Masaki, GOTOH Akihiko, KOMATSU Norio

doi:10.11406/rinketsu.57.987

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重篤な心機能障害を伴う未治療ALアミロイドーシスに対するbortezomibの有用性
臨床研究重篤な心機能障害を伴う未治療ALアミロイドーシスに対するbortezomibの有用性
リンショウケンキュウジュウトクナシンキノウショウガイオトモナウミチリョウ AL アミロイドーシスニタイスル bortezomib ノユウヨウセイ

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<p>Cardiac amyloid light-chain amyloidosis (AL amyloidosis) is a rare disease with a very poor prognosis, associated with plasma cell dyscrasias such as monoclonal gammopathy of undetermined significance and multiple myeloma. Though bortezomib-containing regimens have achieved high hematologic response rates, there are still few reports describing the outcomes of Japanese patients. Six patients with severe cardiac AL amyloidosis were treated with bortezomib-containing regimens. Involved free light chain (iFLC) decreased immediately in most of these cases. However, the condition of heart failure and N-terminal pro-B-type natriuretic peptide (NT-proBNP) worsened in the early phase of this treatment and then improved several months later. At 29 months, the median duration of follow-up (2-47months), all patients remain alive except one who died of sudden cardiac arrest. Bortezomib-containing regimens are considered to be among the effective treatments for severe cardiac AL amyloidosis.</p>

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Rinsho Ketsueki

Rinsho Ketsueki 57 (8), 987-993, 2016

The Japanese Society of Hematology

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