Cargando…
Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis
BACKGROUND: Amyloidosis is a systemic disorder that results from the tissue deposition of various proteins with distinctive morphological characteristics. Conjunctival amyloidosis is a rare variant which is generally localized and not associated with systemic involvement. CASE REPORT: We present her...
Autores principales: | , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
International Scientific Literature, Inc.
2012
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3615912/ https://www.ncbi.nlm.nih.gov/pubmed/23569501 http://dx.doi.org/10.12659/AJCR.883022 |
_version_ | 1782265062756450304 |
---|---|
author | Abdallah, Al-Ola Westfall, Christopher Brown, Harry Muzaffar, Jameel Atrash, Shebli Nair, Bijay |
author_facet | Abdallah, Al-Ola Westfall, Christopher Brown, Harry Muzaffar, Jameel Atrash, Shebli Nair, Bijay |
author_sort | Abdallah, Al-Ola |
collection | PubMed |
description | BACKGROUND: Amyloidosis is a systemic disorder that results from the tissue deposition of various proteins with distinctive morphological characteristics. Conjunctival amyloidosis is a rare variant which is generally localized and not associated with systemic involvement. CASE REPORT: We present here a case of 47-year-old female patient with right eyelid swelling that progressed over a 12 year period and eventually underwent surgery with pathology showing AL conjunctival amyloidosis. Unlike in most other reported cases of localized amyloidosis, she was noted to have amyloid deposition in the bone marrow and gastrointestinal tract upon extensive evaluation without any evidence of underlying plasma cell dyscrasia. She has been on observation without evidence of systemic progression or recurrence of conjunctival amyloid. CONCLUSIONS: Although it initially appeared that our case represented an isolated form of AL (kappa)-type conjunctival amyloidosis, systemic evaluation revealed trace amount of amyloid in the bone marrow and GI tract. It is feasible that upon very close scrutiny patients with seemingly localized AL amyloidosis may have trace amounts of amyloid involving other organs and based on experience from this single patient we believe that it is safe to observe such patients closely rather than pursue systemic therapy |
format | Online Article Text |
id | pubmed-3615912 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | International Scientific Literature, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-36159122013-04-08 Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis Abdallah, Al-Ola Westfall, Christopher Brown, Harry Muzaffar, Jameel Atrash, Shebli Nair, Bijay Am J Case Rep Case Report BACKGROUND: Amyloidosis is a systemic disorder that results from the tissue deposition of various proteins with distinctive morphological characteristics. Conjunctival amyloidosis is a rare variant which is generally localized and not associated with systemic involvement. CASE REPORT: We present here a case of 47-year-old female patient with right eyelid swelling that progressed over a 12 year period and eventually underwent surgery with pathology showing AL conjunctival amyloidosis. Unlike in most other reported cases of localized amyloidosis, she was noted to have amyloid deposition in the bone marrow and gastrointestinal tract upon extensive evaluation without any evidence of underlying plasma cell dyscrasia. She has been on observation without evidence of systemic progression or recurrence of conjunctival amyloid. CONCLUSIONS: Although it initially appeared that our case represented an isolated form of AL (kappa)-type conjunctival amyloidosis, systemic evaluation revealed trace amount of amyloid in the bone marrow and GI tract. It is feasible that upon very close scrutiny patients with seemingly localized AL amyloidosis may have trace amounts of amyloid involving other organs and based on experience from this single patient we believe that it is safe to observe such patients closely rather than pursue systemic therapy International Scientific Literature, Inc. 2012-06-06 /pmc/articles/PMC3615912/ /pubmed/23569501 http://dx.doi.org/10.12659/AJCR.883022 Text en © Am J Case Rep, 2012 This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License. |
spellingShingle | Case Report Abdallah, Al-Ola Westfall, Christopher Brown, Harry Muzaffar, Jameel Atrash, Shebli Nair, Bijay Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title | Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title_full | Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title_fullStr | Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title_full_unstemmed | Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title_short | Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis |
title_sort | unilateral conjunctival al kappa amyloidosis with trace evidence of systemic amyloidosis |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3615912/ https://www.ncbi.nlm.nih.gov/pubmed/23569501 http://dx.doi.org/10.12659/AJCR.883022 |
work_keys_str_mv | AT abdallahalola unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis AT westfallchristopher unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis AT brownharry unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis AT muzaffarjameel unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis AT atrashshebli unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis AT nairbijay unilateralconjunctivalalkappaamyloidosiswithtraceevidenceofsystemicamyloidosis |