Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.

dc.contributor.author

Yu, Li

dc.contributor.author

Tu, Meifeng

dc.contributor.author

Cortes, Jorge

dc.contributor.author

Xu-Monette, Zijun Y

dc.contributor.author

Miranda, Roberto N

dc.contributor.author

Zhang, Jun

dc.contributor.author

Orlowski, Robert Z

dc.contributor.author

Neelapu, Sattva

dc.contributor.author

Boddu, Prajwal C

dc.contributor.author

Akosile, Mary A

dc.contributor.author

Uldrick, Thomas S

dc.contributor.author

Yarchoan, Robert

dc.contributor.author

Medeiros, L Jeffrey

dc.contributor.author

Li, Yong

dc.contributor.author

Fajgenbaum, David C

dc.contributor.author

Young, Ken H

dc.date.accessioned

2019-09-21T21:25:27Z

dc.date.available

2019-09-21T21:25:27Z

dc.date.issued

2017-03

dc.date.updated

2019-09-21T21:25:26Z

dc.description.abstract

Castleman disease (CD) comprises 3 poorly understood lymphoproliferative variants sharing several common histopathological features. Unicentric CD (UCD) is localized to a single region of lymph nodes. Multicentric CD (MCD) manifests with systemic inflammatory symptoms and organ dysfunction due to cytokine dysregulation and involves multiple lymph node regions. Human herpesvirus 8 (HHV-8) causes MCD (HHV-8-associated MCD) in immunocompromised individuals, such as HIV-infected patients. However, >50% of MCD cases are HIV and HHV-8 negative (defined as idiopathic [iMCD]). The clinical and biological behavior of CD remains poorly elucidated. Here, we analyzed the clinicopathologic features of 74 patients (43 with UCD and 31 with iMCD) and therapeutic response of 96 patients (43 with UCD and 53 with iMCD) with HIV-/HHV-8-negative CD compared with 51 HIV-/HHV-8-positive patients. Systemic inflammatory symptoms and elevated inflammatory factors were more common in iMCD patients than UCD patients. Abnormal bone marrow features were more frequent in iMCD (77.0%) than UCD (45%); the most frequent was plasmacytosis, which was seen in 3% to 30.4% of marrow cells. In the lymph nodes, higher numbers of CD3+ lymphocytes (median, 58.88 ± 20.57) and lower frequency of CD19+/CD5+ (median, 5.88 ± 6.52) were observed in iMCD patients compared with UCD patients (median CD3+ cells, 43.19 ± 17.37; median CD19+/CD5+ cells, 17.37 ± 15.80). Complete surgical resection is a better option for patients with UCD. Siltuximab had a greater proportion of complete responses and longer progression-free survival (PFS) for iMCD than rituximab. Centricity, histopathological type, and anemia significantly impacted PFS. This study reveals that CD represents a heterogeneous group of diseases with differential immunophenotypic profiling and treatment response.

dc.identifier

blood-2016-11-748855

dc.identifier.issn

0006-4971

dc.identifier.issn

1528-0020

dc.identifier.uri

https://hdl.handle.net/10161/19333

dc.language

eng

dc.publisher

American Society of Hematology

dc.relation.ispartof

Blood

dc.relation.isversionof

10.1182/blood-2016-11-748855

dc.subject

Humans

dc.subject

Herpesvirus 8, Human

dc.subject

HIV-1

dc.subject

Inflammation

dc.subject

Antibodies, Monoclonal

dc.subject

Disease-Free Survival

dc.subject

Immunophenotyping

dc.subject

Adolescent

dc.subject

Adult

dc.subject

Aged

dc.subject

Middle Aged

dc.subject

Female

dc.subject

Male

dc.subject

Young Adult

dc.subject

Castleman Disease

dc.title

Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.

dc.type

Journal article

duke.contributor.orcid

Xu-Monette, Zijun Y|0000-0002-7615-3949

duke.contributor.orcid

Young, Ken H|0000-0002-5755-8932

pubs.begin-page

1658

pubs.end-page

1668

pubs.issue

12

pubs.organisational-group

School of Medicine

pubs.organisational-group

Duke

pubs.organisational-group

Pathology

pubs.organisational-group

Clinical Science Departments

pubs.publication-status

Published

pubs.volume

129

Files

Original bundle

Now showing 1 - 1 of 1
Loading...
Thumbnail Image
Name:
149, Yu Li, MCD Blood 2017.pdf
Size:
2.1 MB
Format:
Adobe Portable Document Format