Literature DB >> 29991549

Multisystem amyloidosis as the unifying diagnosis for constipation, collapse and cardiomyopathy.

Michael McFarlane1, Alexander Bashford1, Shatrughan Sah2, Ben R Disney1.   

Abstract

Amyloidosis a rare disorder characterised by the deposition of amyloid protein aggregates in different organ systems throughout the body with resulting functional impairment of affected organs. It can present with localised or multisystemic deposits. Diagnosis is often delayed due to the non-specific nature of the symptoms. We present the case of a 59-year-old man with a 12-month history of non-specific symptoms. Investigations revealed Helicobacter pylori positive gastritis. Blood tests showed only a normocytic anaemia and thrombocytopaenia. CT scan showed proximal sigmoid thickening. Biopsies were unremarkable. Echocardiogram and cardiac MRI scan showed restrictive cardiomyopathy. Congo red staining of gastric biopsies showed amyloid deposition. The patient had elevated serum kappa light chains and a bone marrow biopsy confirmed multiple myeloma and he was subsequently diagnosed with systemic light chain (AL) amyloidosis secondary to this. He was started on chemotherapy and parenteral nutrition; however, he deteriorated rapidly and so was started on palliative treatment and discharged home. © BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.

Entities:  

Keywords:  haematology (incl blood transfusion); malabsorption; pericardial disease; stomach and duodenum

Mesh:

Substances:

Year:  2018        PMID: 29991549      PMCID: PMC6047702          DOI: 10.1136/bcr-2018-225301

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  9 in total

1.  Endoscopic and histopathological features of gastrointestinal amyloidosis.

Authors:  Akira Hokama; Kazuto Kishimoto; Manabu Nakamoto; Chiharu Kobashigawa; Tetsuo Hirata; Nagisa Kinjo; Fukunori Kinjo; Seiya Kato; Jiro Fujita
Journal:  World J Gastrointest Endosc       Date:  2011-08-16

2.  Guidelines on the diagnosis and investigation of AL amyloidosis.

Authors:  Julian D Gillmore; Ashutosh Wechalekar; Jenny Bird; Jamie Cavenagh; Stephen Hawkins; Majid Kazmi; Helen J Lachmann; Philip N Hawkins; Guy Pratt
Journal:  Br J Haematol       Date:  2014-10-14       Impact factor: 6.998

3.  A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis.

Authors:  Ashutosh D Wechalekar; Stefan O Schonland; Efstathios Kastritis; Julian D Gillmore; Meletios A Dimopoulos; Thirusha Lane; Andrea Foli; Darren Foard; Paolo Milani; Lisa Rannigan; Ute Hegenbart; Philip N Hawkins; Giampaolo Merlini; Giovanni Palladini
Journal:  Blood       Date:  2013-03-11       Impact factor: 22.113

4.  Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience.

Authors:  Andrew J Cowan; Martha Skinner; David C Seldin; John L Berk; David R Lichtenstein; Carl J O'Hara; Gheorghe Doros; Vaishali Sanchorawala
Journal:  Haematologica       Date:  2012-06-24       Impact factor: 9.941

5.  Guidelines on the management of AL amyloidosis.

Authors:  Ashutosh D Wechalekar; Julian D Gillmore; Jenny Bird; Jamie Cavenagh; Stephen Hawkins; Majid Kazmi; Helen J Lachmann; Philip N Hawkins; Guy Pratt
Journal:  Br J Haematol       Date:  2014-10-10       Impact factor: 6.998

6.  Clinical features and treatment response of light chain (AL) amyloidosis diagnosed in patients with previous diagnosis of multiple myeloma.

Authors:  Sumit Madan; Angela Dispenzieri; Martha Q Lacy; Francis Buadi; Suzanne R Hayman; Steven R Zeldenrust; S Vincent Rajkumar; Morie A Gertz; Shaji K Kumar
Journal:  Mayo Clin Proc       Date:  2010-03       Impact factor: 7.616

Review 7.  Update on treatment of light chain amyloidosis.

Authors:  Shameem Mahmood; Giovanni Palladini; Vaishali Sanchorawala; Ashutosh Wechalekar
Journal:  Haematologica       Date:  2014-02       Impact factor: 9.941

8.  Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis.

Authors:  Angela Dispenzieri; Morie A Gertz; Robert A Kyle; Martha Q Lacy; Mary F Burritt; Terry M Therneau; Philip R Greipp; Thomas E Witzig; John A Lust; S Vincent Rajkumar; Rafael Fonseca; Steven R Zeldenrust; Christopher G A McGregor; Allan S Jaffe
Journal:  J Clin Oncol       Date:  2004-09-15       Impact factor: 44.544

Review 9.  Al amyloidosis.

Authors:  Estelle Desport; Frank Bridoux; Christophe Sirac; Sébastien Delbes; Sébastien Bender; Béatrice Fernandez; Nathalie Quellard; Corinne Lacombe; Jean-Michel Goujon; David Lavergne; Julie Abraham; Guy Touchard; Jean-Paul Fermand; Arnaud Jaccard
Journal:  Orphanet J Rare Dis       Date:  2012-08-21       Impact factor: 4.123

  9 in total

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