Hepatic, gastric and bone marrow AL amyloidosis that began with Budd-Chiari syndrome: a case report.

Autor: Manieri VM; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Offidani M; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Capelli D; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Marzioni M; Clinica di Gastroenterologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy.; Università Politecnica delle Marche, Facoltà Medicina e Chirurgia, Ancona, Italia., Maroni L; Clinica di Gastroenterologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Filosa A; Anatomia Patologica, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Rupoli S; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Morsia E; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Poloni A; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy., Morè S; Clinica di Ematologia, Azienda Ospedaliero Universitaria delle Marche, Ancona, Italy. s.more@staff.univpm.it.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2024 Sep; Vol. 103 (9), pp. 3783-3786. Date of Electronic Publication: 2024 Jun 14.
DOI: 10.1007/s00277-024-05837-2
Abstrakt: Amyloid Light Chain (AL) Amyloidosis is a rare disorder of protein misfolding and metabolism characterized by insoluble fibrils deposition in various tissues and organs, which could quickly progress and become fatal. The most frequently affected organ is heart being its involvement the most adverse prognostic feature. Kidney and liver could be other organ localizations, defining AL Amyloidosis as a multisystem disorder. Being Budd-Chiari syndrome (BCS) an uncommon congestive hepatopathy caused by blockage of hepatic veins in the absence of cardiac disorders, it could be rarely caused by a massive deposition of amyloid proteins into hepatic sinusoidal spaces, giving an uncommon clinical presentation of AL Amyloidosis.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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