Comprehensive analysis of 65 patients with Castleman disease in a single center in China.

This study aimed to investigate the epidemiologic, clinical, pathological characteristics, and treatment of patients with Castleman disease (CD) in a single center in China. We retrospectively analyzed the data of 65 Chinese CD patients, divided into unicentric CD (UCD) and multicentric CD (MCD) groups, and also microscopic subtypes as hypervascular (HV), plasmacytic (PC) and Mixed. Based on whether HHV-8 infection existed, MCD was subdivided into HHV-8-associated MCD and idiopathic Castleman disease (iMCD). Detailed epidemiologic, clinicopathological, and treatment data were analyzed and discussed. Of total 65 patients (UCD 33, MCD 32), HV (81.8%) accounted for the most of UCD and total. More females in UCD (60.6%) and more males in MCD (65.6%) were observed. CD occurred in all age groups, most commonly in 40-49 years. The mean age of onset of total was 38.5 years with PC higher than HV (45.5 vs. 35.1 years, P = 0.0413). The median diagnosis delay of MCD was longer than that of UCD (3.00 vs. 1.25 months, P = 0.0436). Abdomen (39.4%) and neck (30.3%) were the most-seen locations of lymphadenopathy in UCD, with neck (65.6%) being predominant in MCD. Mean major diameter of specimens of UCD was greater than MCD (6.4 vs. 3.1 cm, P < 0.0001). These results provided the featured and detailed profile of Castleman disease in Henan province in China with a considerable number of cases, which presented distinct evidence with other studies.

Wang XQ ，Zhong NN ，Sun Q ，Yan SC ，Xu GC ，Wang YG ，Peng LW ，Liu B ，Bu LL ... -  《Scientific Reports》

Virome capture sequencing does not identify active viral infection in unicentric and idiopathic multicentric Castleman disease.

Nabel CS ，Sameroff S ，Shilling D ，Alapat D ，Ruth JR ，Kawano M ，Sato Y ，Stone K ，Spetalen S ，Valdivieso F ，Feldman MD ，Chadburn A ，Fosså A ，van Rhee F ，Lipkin WI ，Fajgenbaum DC ... -  《PLoS One》

Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital.

González-García A ，Patier de la Peña JL ，García-Cosio M ，Sarhane Y ，Sánchez Díaz C ，Barbolla Díaz I ，López Rodríguez M ，Moreno MÁ ，Villarubia J ，Manzano L ... -  《-》

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two-site retrospective cohort study.

Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus-8 (HHV8)-negative multicentric CD (MCD), in a multi-institutional cohort. We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals. Demographic and clinical data were collected. Most patients (75%, 18/24) presented with UCD. All patients with MCD were HHV8-negative. The most common histopathologic variant was hyaline vascular (75%, 18/24). Plasma cell variant occurred in 33% (2/6 [95% confidence intervals (CI), 4-78%]) of patients with HHV8-negative MCD and 17% (3/18 [95% CI, 4-41%]) of patients with UCD. Systemic symptoms were present in 4 of 6 of patients with HHV8-negative MCD and 8 of 18 of patients with UCD. Anemia and laboratory inflammation occurred in both UCD and MCD patients, with nonsignificantly higher rates of anemia and elevated C-reactive protein in MCD patients. All but two UCD patients underwent gross total resection as definitive therapy. Among HHV8-negative MCD patients, a combination of resection, chemotherapy, and immunotherapy was used. No UCD patients and three of six HHV8-negative MCD patients experienced disease progression/relapse prior to lasting remission. There were no deaths. Pediatric patients with CD most commonly have unicentric, hyaline vascular variant disease. Pediatric patients with both UCD and MCD commonly have systemic inflammation and, despite risk of progression/relapse in MCD patients, ultimately have excellent survival.

Sopfe J ，Endres A ，Campbell K ，Hayes K ，Trout AT ，Liang X ，Lorsbach R ，O'Brien MM ，Cost CR ... -  《-》

The clinical picture of Castleman disease: a systematic review and meta-analysis.

Castleman disease (CD) encompasses a spectrum of rare disorders, including unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD). We performed a systematic review of publications reporting ≥5 cases of CD between 1995 and 2021, following preferred reporting items for systematic reviews and meta-analyses guidelines, to describe and compare subtypes. We extracted data on clinical symptoms and laboratory parameters as stated in international consensus diagnostic criteria for iMCD and estimated the frequency of each criterion using meta-analyses. We analyzed 32 studies describing 559 UCD, 1023 iMCD, and 416 HHV8+ MCD cases. Although many symptoms and laboratory abnormalities occurred at similar rates in patients with iMCD and HHV8+ MCD, patients with HHV8+ MCD had significantly higher rates of constitutional symptoms (46.6% vs 98.6%; P = .038) and splenomegaly (48.2% vs 89.2%; P = .031). Renal dysfunction was significantly more common in patients with iMCD than in patients with HHV8+ MCD before adjustment (36.9% vs 17.4%; P = .04; adjusted P = .1). Patients with UCD had lower rates of symptoms and laboratory abnormalities, although these were present in 20% of patients and were particularly pronounced in pediatric UCD. There are many similarities in the symptomatology of iMCD and HHV8+ MCD; many patients experience constitutional symptoms and organ dysfunction. Differences between these subtypes likely reflect differences in pathophysiology and/or comorbidity burdens.

Hoffmann C ，Oksenhendler E ，Littler S ，Grant L ，Kanhai K ，Fajgenbaum DC ... -  《-》