Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.
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.
Type
Journal articleSubject
HumansHerpesvirus 8, Human
HIV-1
Inflammation
Antibodies, Monoclonal
Disease-Free Survival
Immunophenotyping
Adolescent
Adult
Aged
Middle Aged
Female
Male
Young Adult
Castleman Disease
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https://hdl.handle.net/10161/19333Published Version (Please cite this version)
10.1182/blood-2016-11-748855Publication Info
Yu, Li; Tu, Meifeng; Cortes, Jorge; Xu-Monette, Zijun Y; Miranda, Roberto N; Zhang,
Jun; ... Young, Ken H (2017). Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.
Blood, 129(12). pp. 1658-1668. 10.1182/blood-2016-11-748855. Retrieved from https://hdl.handle.net/10161/19333.This is constructed from limited available data and may be imprecise. To cite this
article, please review & use the official citation provided by the journal.
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Show full item recordScholars@Duke
Zijun Yidan Xu-Monette
Assistant Professor in Pathology
My research efforts have been focused on identifying prognostic and therapeutic biomarkers
in B-cell lymphoma. My research interests also include investigation of molecular
and immune mechanisms underlying the poor clinical outcomes of lymphoma, the pathogenesis
and evolution of drug resistant clones, and development of novel therapies for aggressive
B-cell lymphoma.
Ken H Young
Professor of Pathology
I am a clinically-oriented diagnostic physician with clinical expertise in the pathologic
diagnosis of hematologic cancers including tumors of the bone marrow, lymphoid tissue,
spleen and pre-malignant hematologic conditions. Another area of interest is blood
cancer classification with molecular and genetic profiling. In my research program,
we focus on molecular mechanisms of tumor progression, cell-of-origin, biomarkers,
and novel therapeutic strategies in lymphoma, myeloma and leukemia. In
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