Cargando…
Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS)
Patient: Female, 68 Final Diagnosis: Light chain monoclonal gammopathy of undetermined significance Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Oncology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Amyloidosis is the extracellular tissue deposition of...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
International Scientific Literature, Inc.
2017
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5713495/ https://www.ncbi.nlm.nih.gov/pubmed/29176541 http://dx.doi.org/10.12659/AJCR.907041 |
_version_ | 1783283437459734528 |
---|---|
author | Junejo, Shoaib Ali, Yasir Lubana, Sandeep Singh Tuli, Sandeep S. |
author_facet | Junejo, Shoaib Ali, Yasir Lubana, Sandeep Singh Tuli, Sandeep S. |
author_sort | Junejo, Shoaib |
collection | PubMed |
description | Patient: Female, 68 Final Diagnosis: Light chain monoclonal gammopathy of undetermined significance Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Oncology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS). Our case is the first case to show two coinciding conditions; one is the association of GIT AL amyloidosis with the incidental finding of a rare type of MGUS (LC-MGUS) and the other is the radiologic presentation of GIT amyloidosis with omental calcification mimicking the GIT malignancy. CASE REPORT: A 68-year-old female presented with symptoms of partial bowel obstruction, including intermittent diffuse abdominal pain and constipation. After computed tomography (CT) abdomen and pelvis, an exploratory laparotomy was needed because of suspicion of abdominal carcinomatosis due to diffuse omental calcification. The tissue sent for biopsy surprisingly showed AL amyloidosis. The patient did not report any systemic symptoms. Further workup was advised to inquire about the plasma cell dyscrasia which eventually turned into a very rare version of MGUS knows as light chain MGUS (LC-MGUS). Following adequate resection of the involved structures, the patient was then placed on chemotherapy and successfully went into remission. CONCLUSIONS: This case report illustrates that in an era of evidence based medicine, it is important to show through case reports the association of GIT AL amyloidosis with LC-MGUS, as the literature on this topic is lacking. It also points to the importance of timely intervention that can greatly enhance, not only the only the chances of remission but also prevention of further complications such as malignant transformation. |
format | Online Article Text |
id | pubmed-5713495 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | International Scientific Literature, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-57134952017-12-07 Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) Junejo, Shoaib Ali, Yasir Lubana, Sandeep Singh Tuli, Sandeep S. Am J Case Rep Article Patient: Female, 68 Final Diagnosis: Light chain monoclonal gammopathy of undetermined significance Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Oncology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS). Our case is the first case to show two coinciding conditions; one is the association of GIT AL amyloidosis with the incidental finding of a rare type of MGUS (LC-MGUS) and the other is the radiologic presentation of GIT amyloidosis with omental calcification mimicking the GIT malignancy. CASE REPORT: A 68-year-old female presented with symptoms of partial bowel obstruction, including intermittent diffuse abdominal pain and constipation. After computed tomography (CT) abdomen and pelvis, an exploratory laparotomy was needed because of suspicion of abdominal carcinomatosis due to diffuse omental calcification. The tissue sent for biopsy surprisingly showed AL amyloidosis. The patient did not report any systemic symptoms. Further workup was advised to inquire about the plasma cell dyscrasia which eventually turned into a very rare version of MGUS knows as light chain MGUS (LC-MGUS). Following adequate resection of the involved structures, the patient was then placed on chemotherapy and successfully went into remission. CONCLUSIONS: This case report illustrates that in an era of evidence based medicine, it is important to show through case reports the association of GIT AL amyloidosis with LC-MGUS, as the literature on this topic is lacking. It also points to the importance of timely intervention that can greatly enhance, not only the only the chances of remission but also prevention of further complications such as malignant transformation. International Scientific Literature, Inc. 2017-11-25 /pmc/articles/PMC5713495/ /pubmed/29176541 http://dx.doi.org/10.12659/AJCR.907041 Text en © Am J Case Rep, 2017 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) ) |
spellingShingle | Article Junejo, Shoaib Ali, Yasir Lubana, Sandeep Singh Tuli, Sandeep S. Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title | Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title_full | Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title_fullStr | Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title_full_unstemmed | Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title_short | Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS) |
title_sort | diffuse peritoneal and bowel wall infiltration by light chain-al amyloidosis with omental calcification mimicking abdominal carcinomatosis – an elderly female with incidental finding of light chain monoclonal gammopathy of undetermined significance (lc-mgus) |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5713495/ https://www.ncbi.nlm.nih.gov/pubmed/29176541 http://dx.doi.org/10.12659/AJCR.907041 |
work_keys_str_mv | AT junejoshoaib diffuseperitonealandbowelwallinfiltrationbylightchainalamyloidosiswithomentalcalcificationmimickingabdominalcarcinomatosisanelderlyfemalewithincidentalfindingoflightchainmonoclonalgammopathyofundeterminedsignificancelcmgus AT aliyasir diffuseperitonealandbowelwallinfiltrationbylightchainalamyloidosiswithomentalcalcificationmimickingabdominalcarcinomatosisanelderlyfemalewithincidentalfindingoflightchainmonoclonalgammopathyofundeterminedsignificancelcmgus AT lubanasandeepsingh diffuseperitonealandbowelwallinfiltrationbylightchainalamyloidosiswithomentalcalcificationmimickingabdominalcarcinomatosisanelderlyfemalewithincidentalfindingoflightchainmonoclonalgammopathyofundeterminedsignificancelcmgus AT tulisandeeps diffuseperitonealandbowelwallinfiltrationbylightchainalamyloidosiswithomentalcalcificationmimickingabdominalcarcinomatosisanelderlyfemalewithincidentalfindingoflightchainmonoclonalgammopathyofundeterminedsignificancelcmgus |