Cargando…
Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience
BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2...
Autores principales: | , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis
2014
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4278007/ https://www.ncbi.nlm.nih.gov/pubmed/25548759 http://dx.doi.org/10.5045/br.2014.49.4.253 |
_version_ | 1782350451273891840 |
---|---|
author | Seo, Seyoung Yoo, Changhoon Yoon, Dok Hyun Kim, Shin Park, Jung Sun Park, Chan-Sik Huh, Jooryung Suh, Cheolwon |
author_facet | Seo, Seyoung Yoo, Changhoon Yoon, Dok Hyun Kim, Shin Park, Jung Sun Park, Chan-Sik Huh, Jooryung Suh, Cheolwon |
author_sort | Seo, Seyoung |
collection | PubMed |
description | BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. RESULTS: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. CONCLUSION: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors. |
format | Online Article Text |
id | pubmed-4278007 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis |
record_format | MEDLINE/PubMed |
spelling | pubmed-42780072014-12-29 Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience Seo, Seyoung Yoo, Changhoon Yoon, Dok Hyun Kim, Shin Park, Jung Sun Park, Chan-Sik Huh, Jooryung Suh, Cheolwon Blood Res Original Article BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. RESULTS: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. CONCLUSION: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors. Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 2014-12 2014-12-23 /pmc/articles/PMC4278007/ /pubmed/25548759 http://dx.doi.org/10.5045/br.2014.49.4.253 Text en © 2014 Korean Society of Hematology http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Seo, Seyoung Yoo, Changhoon Yoon, Dok Hyun Kim, Shin Park, Jung Sun Park, Chan-Sik Huh, Jooryung Suh, Cheolwon Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title | Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title_full | Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title_fullStr | Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title_full_unstemmed | Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title_short | Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience |
title_sort | clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric castleman's disease: a single medical center experience |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4278007/ https://www.ncbi.nlm.nih.gov/pubmed/25548759 http://dx.doi.org/10.5045/br.2014.49.4.253 |
work_keys_str_mv | AT seoseyoung clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT yoochanghoon clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT yoondokhyun clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT kimshin clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT parkjungsun clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT parkchansik clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT huhjooryung clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience AT suhcheolwon clinicalfeaturesandoutcomesinpatientswithhumanimmunodeficiencyvirusnegativemulticentriccastlemansdiseaseasinglemedicalcenterexperience |