Clinical features and treatment outcomes of Castleman disease in children: a retrospective cohort in China.

Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children's Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression. Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes. • Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics. • The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics. • We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease. • Our work may improve the recognition and optimize the management of this rare disease in children.

Hu S ，Li Z ，Wang H ，Chen L ，Ma Y ，Zhu X ，Li J ，Dong R ，Yao W ，Dong C ，Zhang H ，Li K ，Dong K ，Zhai X ... -  《-》

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 ... -  《-》

Castleman Disease: A Multicenter Case Series from Turkey.

Gündüz E ，Kırkızlar HO ，Ümit EG ，Karaman Gülsaran S ，Özkocaman V ，Özkalemkaş F ，Candar Ö ，Elverdi T ，Küçükyurt S ，Paydaş S ，Çeneli Ö ，Karakuş S ，Maral S ，Ekinci Ö ，İpek Y ，Kis C ，Güven ZT ，Akdeniz A ，Celkan T ，Eroğlu Küçükdiler AH ，Akgün Çağlıyan G ，Özçelik Şengöz C ，Karataş A ，Bulduk T ，Özcan A ，Belen Apak FB ，Canbolat A ，Kartal İ ，Ören H ，Töret E ，Özdemir GN ，Bakanay Öztürk ŞM ... -  《-》

The French paediatric cohort of Castleman disease: a retrospective report of 23 patients.

Borocco C ，Ballot-Schmit C ，Ackermann O ，Aladjidi N ，Delaleu J ，Giacobbi-Milet V ，Jannier S ，Jeziorski E ，Maurier F ，Perel Y ，Piguet C ，Oksenhendler E ，Koné-Paut I ，Galeotti C ... -  《Orphanet Journal of Rare Diseases》

[Clinical Features and Prognosis of Patients with Castleman's Disease].

Huang XJ ，Zhang XL ，Wei XF ，Liang XQ ，Fu Y ，Zhao YY ，Li QF ，Zhang QK ，Feng YF ... -  《-》