Abstract
Castleman’s disease (CD) is a rare lymphoproliferative disorder with clinical features and prognostic factors that are incompletely characterized. This retrospective single-center study reviewed the largest HIV-negative CD patient cohort (n = 145) to date. By clinical classification, we identified 69 patients (47.6%) as unicentric CD (UCD) and 76 patients (52.4%) as multicentric CD (MCD). Pathological classification identified 74 patients (51.0%) with the hyaline-vascular variant, 51 patients (35.2%) with the plasma-cell variant, and 20 patients (13.8%) with a mixed variant. After a median follow-up duration of 58 months (range, 1–180 months), the 1-year and 5-year survival rates were 95.1% and 91.0%, respectively. UCD patients exhibited significantly better survival (1-year and 5-year survival rates of 98.5% and 97.1%, respectively) compared with MCD patients (1-year and 5-year survival rates of 92.1% and 85.5%, respectively; p = 0.005). By univariate and multivariate Cox regression analyses, the estimated glomerular filtration rate < 60 ml/min (with the MDRD equation; hazard ratio = 4.60; 95% confidence interval, 1.50–14.12; p = 0.008) was clinically significant and represented an independent predictor for death in MCD patients. In summary, this large-scale study suggests that UCD patients enjoy better survival than MCD patients and that renal function is an important prognostic factor for MCD patients.
Highlights
Castleman’s disease (CD, angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder that was first described in 1956 by Benjamin Castleman and colleagues[1]
A total of 145 human immunodeficiency virus (HIV)-negative CD patients were enrolled in this study, including 69 (47.6%) males and 76 (52.4%) females
After incorporating variables with P < 0.10 in univariate analysis, multivariate Cox regression (Table 1) revealed that only estimated glomerular filtration rate (eGFR) < 60 ml/min (HR = 4.60; 95% confidence interval (CI), 1.50–14.12; p = 0.008) was a clinically significant and independent predictor for death in multicentric CD (MCD) patients (Fig. 2)
Summary
Castleman’s disease (CD, angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder that was first described in 1956 by Benjamin Castleman and colleagues[1]. Because of the rarity of CD, knowledge about its clinical characteristics has accumulated slowly, and is mostly described in case reports[3]. Human immunodeficiency virus (HIV) status is recognized to be an important factor in patients with CD9, previous studies have enrolled both HIV-positive and HIV-negative patients[3,4] (as well as patients with an ambiguous HIV status[5,6]). HIV status itself might represent a risk factor for survival in CD patients[4], it may be helpful to perform survival analysis in HIV-negative patients to identify risk factors other than HIV status for CD patients, especially in countries with a low HIV prevalence, such as China[10]. We report 145 HIV-negative CD patients with available clinical and pathological data; we attempted to delineate the survival pattern and to identify prognostic factors for survival in these patients
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