A Retrospective Analysis of Clinical Features and Treatment Outcome in 21 Patients with Immunoglobulin M-related Light-chain Amyloidosis in Japan: a Study from the Amyloidosis Research Committee

Overview

Journal Int J Hematol

Specialty Hematology

Date 2023 Jul 29

PMID 37515656

Authors

Shin-Ichi Fuchida

Mizuki Ogura

Tadao Ishida

Hiroyuki Hata

Hiroshi Handa

Nagaaki Katoh

Chiaki Nakaseko

Kazutaka Sunami

Yuta Katayama

Hironobu Nobata

Kazuiku Oshiro

Shinsuke Iida

Yoshiki Sekijima

Hironobu Naiki

Chihiro Shimazaki

Affiliations

Soon will be listed here.

Abstract

We retrospectively gathered data of 21 patients (13 male and 8 female; median age 65 years) diagnosed with immunoglobulin M (IgM)-related light-chain (AL) amyloidosis in Japan to investigate characteristics of IgM-AL amyloidosis and its optimal treatment strategy. Median IgM and difference free light chain (FLC) at diagnosis were 1257 mg/dl and 34.3 mg/l, respectively. Organ involvement was observed in the heart in 7 patients (33%), kidneys in 15 (71%), and lymph nodes in 5 (24%). Initial treatments were melphalan/dexamethasone in 7 patients, bortezomib/cyclophosphamide/dexamethasone in 3, autologous stem cell transplantation in 3, rituximab/bendamustine in 1, other in 3, and none in 4. Hematological responses among 15 evaluable patients were as follows: 3 reached complete response (CR), 4 partial response (PR), and 1 very good PR (VGPR), making the overall response rate of PR or better 40%. Median overall survival (OS) was 14.0 months and 1-year OS was 71.4%. Prognosis was significantly poorer in patients with cardiac involvement than those with non-cardiac involvement (1-year OS 27.8% vs. 85.7%, p = 0.0468). The involved FLC value was low in several patients and therapeutic response was difficult to assess. Further study is necessary to determine the optimal treatment for IgM-AL amyloidosis.

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