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

Publication ,  Journal Article
Yu, L; Tu, M; Cortes, J; Xu-Monette, ZY; Miranda, RN; Zhang, J; Orlowski, RZ; Neelapu, S; Boddu, PC; Akosile, MA; Uldrick, TS; Yarchoan, R ...
Published in: Blood
March 2017

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.

Duke Scholars

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Published In

Blood

DOI

EISSN

1528-0020

ISSN

0006-4971

Publication Date

March 2017

Volume

129

Issue

12

Start / End Page

1658 / 1668

Related Subject Headings

  • Young Adult
  • Middle Aged
  • Male
  • Inflammation
  • Immunophenotyping
  • Immunology
  • Humans
  • Herpesvirus 8, Human
  • HIV-1
  • Female
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Yu, L., Tu, M., Cortes, J., Xu-Monette, Z. Y., Miranda, R. N., Zhang, J., … Young, K. H. (2017). Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood, 129(12), 1658–1668. https://doi.org/10.1182/blood-2016-11-748855
Yu, Li, Meifeng Tu, Jorge Cortes, Zijun Y. Xu-Monette, Roberto N. Miranda, Jun Zhang, Robert Z. Orlowski, et al. “Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.Blood 129, no. 12 (March 2017): 1658–68. https://doi.org/10.1182/blood-2016-11-748855.
Yu L, Tu M, Cortes J, Xu-Monette ZY, Miranda RN, Zhang J, et al. Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood. 2017 Mar;129(12):1658–68.
Yu, Li, et al. “Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease.Blood, vol. 129, no. 12, Mar. 2017, pp. 1658–68. Epmc, doi:10.1182/blood-2016-11-748855.
Yu L, Tu M, Cortes J, Xu-Monette ZY, Miranda RN, Zhang J, Orlowski RZ, Neelapu S, Boddu PC, Akosile MA, Uldrick TS, Yarchoan R, Medeiros LJ, Li Y, Fajgenbaum DC, Young KH. Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood. 2017 Mar;129(12):1658–1668.

Published In

Blood

DOI

EISSN

1528-0020

ISSN

0006-4971

Publication Date

March 2017

Volume

129

Issue

12

Start / End Page

1658 / 1668

Related Subject Headings

  • Young Adult
  • Middle Aged
  • Male
  • Inflammation
  • Immunophenotyping
  • Immunology
  • Humans
  • Herpesvirus 8, Human
  • HIV-1
  • Female