PMID- 33264681
OWN - NLM
STAT- MEDLINE
DCOM- 20210608
LR  - 20210608
IS  - 1879-1336 (Electronic)
IS  - 1054-8807 (Linking)
VI  - 51
DP  - 2021 Mar-Apr
TI  - Coexistence of amyloidosis and light chain deposition disease in the heart.
PG  - 107315
LID - S1054-8807(20)30119-8 [pii]
LID - 10.1016/j.carpath.2020.107315 [doi]
AB  - There are few reports on the coexistence of cardiac amyloid light-chain (AL) 
      amyloidosis and light chain deposition disease (LCDD), despite their similar 
      pathophysiologies caused by plasma-cell dyscrasia. Herein, we report the 
      coexistence of these diseases. A 59-year-old man was referred to our hospital 
      because of exertional dyspnea and hypotension. Renal dysfunction of unknown 
      etiology had been present for 4 years and hemodialysis had been introduced. 
      Severe systolic and diastolic cardiac dysfunction was apparent, accompanied with 
      dilatation and granular sparkling, but not with left ventricular hypertrophy. The 
      plasma-free light chain kappa was found to be extremely high, with a kappa/lambda ratio of 
      1,919. Light microscopic examination of the endomyocardial biopsy revealed spotty 
      and homogenous deposits, which positively stained with Congo red, and exhibited a 
      blazing apple-green color under polarized light. Based on these results, cardiac 
      amyloidosis was diagnosed. In specimens prepared for electron microscopy, no 
      amyloid fibrils could be found. Instead, we observed amorphous nonfibrillar 
      deposits around several small vessels including capillaries and small arteries, 
      which were consistent with light-chain deposits. LCDD was diagnosed based on the 
      systemic increase in kappa light chain and the ultrastructural findings of the 
      endomyocardial biopsy specimens. Coexistence of cardiac amyloidosis and LCDD was 
      thus confirmed in our patient. An electron microscopic assessment in addition to 
      Congo red staining may be useful to diagnose latent LCDD in patients with 
      suspected cardiac light-chain amyloidosis.
CI  - Copyright (c) 2020. Published by Elsevier Inc.
FAU - Sobue, Yoshihiro
AU  - Sobue Y
AD  - Department of Cardiovascular Medicine, Gifu Heart Center, Gifu, Japan. Electronic 
      address: sobue@heart-center.or.jp.
FAU - Takemura, Genzou
AU  - Takemura G
AD  - Department of Internal Medicine, Asahi University School of Dentistry, Mizuho, 
      Japan.
FAU - Kawamura, Shunji
AU  - Kawamura S
AD  - Department of Pathology, Itabashi Medical Laboratory, EIL Inc., Tokyo, Japan.
FAU - Yano, Toshiyuki
AU  - Yano T
AD  - Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical 
      University School of Medicine, Sapporo, Japan.
FAU - Kanamori, Hiromitsu
AU  - Kanamori H
AD  - Department of Cardiology, Gifu University School of Medicine, Gifu, Japan.
FAU - Morimoto, Shin-Ichiro
AU  - Morimoto SI
AD  - Department of Cardiology, Fujita Health University School of Medicine, Toyoake, 
      Japan.
FAU - Matsuo, Hitoshi
AU  - Matsuo H
AD  - Department of Cardiovascular Medicine, Gifu Heart Center, Gifu, Japan.
LA  - eng
PT  - Case Reports
DEP - 20201129
PL  - United States
TA  - Cardiovasc Pathol
JT  - Cardiovascular pathology : the official journal of the Society for Cardiovascular 
      Pathology
JID - 9212060
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Biopsy
MH  - Cardiomyopathies/immunology/*pathology/therapy
MH  - Fatal Outcome
MH  - Humans
MH  - Immunoglobulin Light Chains/*ultrastructure
MH  - Immunoglobulin Light-chain Amyloidosis/immunology/*pathology/therapy
MH  - Male
MH  - Microscopy, Electron
MH  - Middle Aged
MH  - Myocardium/immunology/*ultrastructure
OTO - NOTNLM
OT  - AL amyloidosis
OT  - Cardiomyopathy
OT  - cardiac amyloidosis
OT  - electron microscopy
OT  - endomyocardial biopsy
OT  - light chain deposition disease
OT  - ultrastructure
EDAT- 2020/12/03 06:00
MHDA- 2021/06/09 06:00
CRDT- 2020/12/02 20:05
PHST- 2020/06/21 00:00 [received]
PHST- 2020/11/24 00:00 [revised]
PHST- 2020/11/25 00:00 [accepted]
PHST- 2020/12/03 06:00 [pubmed]
PHST- 2021/06/09 06:00 [medline]
PHST- 2020/12/02 20:05 [entrez]
AID - S1054-8807(20)30119-8 [pii]
AID - 10.1016/j.carpath.2020.107315 [doi]
PST - ppublish
SO  - Cardiovasc Pathol. 2021 Mar-Apr;51:107315. doi: 10.1016/j.carpath.2020.107315. 
      Epub 2020 Nov 29.