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Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome

A 34-year-old man was referred to the outpatient clinic because of progressive abdominal pain, weight loss and pancytopenia. His body mass index (BMI) had fallen to 14.2 kg/m(2). A CT angiography (CTA) showed narrowing of the truncus coeliacus with poststenotic dilation, and duodenal biopsy revealed...

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Autores principales: Miguelañez Matute, Lorena, van Noord, Desiree, Rayman, Nazik, Guillen, Samara S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8768499/
https://www.ncbi.nlm.nih.gov/pubmed/35039369
http://dx.doi.org/10.1136/bcr-2021-246916
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author Miguelañez Matute, Lorena
van Noord, Desiree
Rayman, Nazik
Guillen, Samara S
author_facet Miguelañez Matute, Lorena
van Noord, Desiree
Rayman, Nazik
Guillen, Samara S
author_sort Miguelañez Matute, Lorena
collection PubMed
description A 34-year-old man was referred to the outpatient clinic because of progressive abdominal pain, weight loss and pancytopenia. His body mass index (BMI) had fallen to 14.2 kg/m(2). A CT angiography (CTA) showed narrowing of the truncus coeliacus with poststenotic dilation, and duodenal biopsy revealed ischaemia establishing a rare diagnosis: median arcuate ligament syndrome (MALS). This explained the postprandial pain and minimal intake. Further pancytopenia workup was performed. The bone marrow displayed gelatinous marrow transformation (GMT), a rare disorder of unknown pathogenesis, which has been associated with severe malnutrition. The final diagnosis was pancytopenia secondary to GMT due to severe malnutrition caused by MALS. The abnormalities in the bone marrow may be reversible by restoring nutritional status. This case emphasises the awareness of GMT in patients with weight loss, malnutrition and cytopenias. To our knowledge, this is the first report demonstrating an association between pancytopenia and MALS.
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spelling pubmed-87684992022-02-04 Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome Miguelañez Matute, Lorena van Noord, Desiree Rayman, Nazik Guillen, Samara S BMJ Case Rep Case Report A 34-year-old man was referred to the outpatient clinic because of progressive abdominal pain, weight loss and pancytopenia. His body mass index (BMI) had fallen to 14.2 kg/m(2). A CT angiography (CTA) showed narrowing of the truncus coeliacus with poststenotic dilation, and duodenal biopsy revealed ischaemia establishing a rare diagnosis: median arcuate ligament syndrome (MALS). This explained the postprandial pain and minimal intake. Further pancytopenia workup was performed. The bone marrow displayed gelatinous marrow transformation (GMT), a rare disorder of unknown pathogenesis, which has been associated with severe malnutrition. The final diagnosis was pancytopenia secondary to GMT due to severe malnutrition caused by MALS. The abnormalities in the bone marrow may be reversible by restoring nutritional status. This case emphasises the awareness of GMT in patients with weight loss, malnutrition and cytopenias. To our knowledge, this is the first report demonstrating an association between pancytopenia and MALS. BMJ Publishing Group 2022-01-17 /pmc/articles/PMC8768499/ /pubmed/35039369 http://dx.doi.org/10.1136/bcr-2021-246916 Text en © BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Case Report
Miguelañez Matute, Lorena
van Noord, Desiree
Rayman, Nazik
Guillen, Samara S
Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title_full Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title_fullStr Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title_full_unstemmed Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title_short Gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
title_sort gelatinous bone marrow transformation due to extensive weight loss in median arcuate ligament syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8768499/
https://www.ncbi.nlm.nih.gov/pubmed/35039369
http://dx.doi.org/10.1136/bcr-2021-246916
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