TY - JOUR
T1 - Clinical and pathological characteristics of HIV-and HHV-8-negative Castleman disease
AU - Yu, Li
AU - Tu, Meifeng
AU - Cortes, Jorge
AU - Xu-Monette, Zijun Y.
AU - Miranda, Roberto N.
AU - Zhang, Jun
AU - Orlowski, Robert Z.
AU - Neelapu, Sattva
AU - Boddu, Prajwal C.
AU - Akosile, Mary A.
AU - Uldrick, Thomas S.
AU - Yarchoan, Robert
AU - Medeiros, L. Jeffrey
AU - Li, Yong
AU - Fajgenbaum, David C.
AU - Young, Ken H.
N1 - Funding Information:
This study is supported by the National Institutes of Health National Cancer Institute (grants R01CA138688, R01CA187415, and 1RC1CA146299) (Y.L. and K.H.Y.) and in part by the Intramural Research Program of the National Institutes of Health National Cancer Institute (T.S.U. and R.Y.). L.Y. and M.T. are the recipients of a Hematology/Oncology Scholarship Award. K.H.Y. is supported by a The University of Texas MD Anderson Cancer Center Institutional Research Grant Award, an Anderson Lymphoma Specialized Programs of Research Excellence (SPORE) Research Development Program Award, an Anderson Myeloma SPORE Research Developmental Program Award, and the University Cancer Foundation via the Sister Institution Network Fund at The University of Texas MD Anderson Cancer Center. The study is also partially supported by the National Institutes of Health, National Cancer Institute grants P50CA136411 and P50CA142509 and an Anderson Cancer Center support grant (CA016672).
Publisher Copyright:
Copyright 2011 by The American Society of Hematology.
PY - 2017/3/23
Y1 - 2017/3/23
N2 - 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 responseof 96patients (43 withUCDand53 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 CD31 lymphocytes (median, 58.88620.57) and lower frequency of CD191/CD51 (median, 5.8866.52) were observed in iMCD patients compared with UCD patients (median CD31 cells, 43.19 6 17.37; median CD191/CD51 cells, 17.37 6 15.80). Complete surgical resection is a better option for patients with UCD. Siltuximab had a greater proportion of complete responses and longer progressionfree 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.
AB - 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 responseof 96patients (43 withUCDand53 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 CD31 lymphocytes (median, 58.88620.57) and lower frequency of CD191/CD51 (median, 5.8866.52) were observed in iMCD patients compared with UCD patients (median CD31 cells, 43.19 6 17.37; median CD191/CD51 cells, 17.37 6 15.80). Complete surgical resection is a better option for patients with UCD. Siltuximab had a greater proportion of complete responses and longer progressionfree 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.
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U2 - 10.1182/blood-2016-11-748855
DO - 10.1182/blood-2016-11-748855
M3 - Article
C2 - 28100459
AN - SCOPUS:85016251572
SN - 0006-4971
VL - 129
SP - 1658
EP - 1668
JO - Blood
JF - Blood
IS - 12
ER -