Castleman's disease is a very rare and potentially severe lymphoproliferative disorder. First sign may be cervical lymphadenitis, requiring sufficient support in diagnosis and therapy by an ENT specialist. Based on a case series the current manuscript gives an overview of the symptoms, the course of disease and the therapy options. Patients with the first diagnosis of a Castleman's disease at the ENT clinic of Ulm University during the years 2011-2015 were included. The duration of symptoms, the applied diagnostic and therapeutic algorithms were evaluated. The duration of the rather weak symptoms was inhomogenous and lasted from 14 days to 14 years. After diagnostic exstirpation a hyaline-vascular type of Castelman's disease was confirmed in all cases. One of the 5 cases proved a multicentric type with an additional axillary manifestation, the others were monocentric. In all patients the diagnostic exstirpation was sufficient for therapy without need for adjuvant medication. At the time of publication all patients are in remission for at least 18 resp. 61 months. The ENT specialist encounters Castleman's disease mostly as a long-lasting swelling of cervical lymph nodes refractory to therapy and without severe concomittant symptoms. Due to potentially unfavorable outcomes a timely diagnostic lymph node exstirpation under general anesthesia is indicated. In most cases this surgical intervention represents the sufficient therapy.
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http://dx.doi.org/10.1055/s-0043-102058 | DOI Listing |
BMC Pediatr
January 2025
Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Nanlishi Road No. 56, Xicheng District, Beijing, 100045, China.
Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of idiopathic multicenter Castleman disease (iMCD)in children.
Methods: From January 2017 to September 2023, basic information, laboratory tests, treatment and prognosis of children diagnosed with iMCD who attended Beijing Children's Hospital of Capital Medical University were collected.
Results: A total of 9 children were enrolled, with a median age of onset of median 11 (2-15) years, 6 males and 3 female.
JAMA Ophthalmol
January 2025
Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Retin Cases Brief Rep
January 2025
Unidade Local de Saúde São José, Lisboa, Portugal MD - Medical Doctor Phone number: +351 965288273 Email:
Purpose: To report a rare case of Multicentric Castleman's Disease presenting with bilateral panuveitis.
Methods: Case report.
Results: A 65-years-old caucasian man presented with progressive blurred vision in both eyes for two weeks, along with weight loss, polyarthralgias and reduced muscle strength persisting for about a year.
Sci Rep
January 2025
Center for Cytokine Storm Treatment & Laboratory, Department of Medicine, University of Pennsylvania, CSTL, 3535 Market Street, Philadelphia, PA, 19104, USA.
Castleman disease (CD) is a rare hematologic disorder characterized by pathologic lymph node changes and a range of symptoms due to excessive cytokine production. While uncontrolled infection with human herpesvirus-8 (HHV-8) is responsible for the cytokine storm in a portion of multicentric CD (HHV-8-associated MCD) cases, the etiology of unicentric CD (UCD) and HHV-8-negative/idiopathic MCD (iMCD) is unknown. Several hypotheses have been proposed regarding the pathogenesis of UCD and iMCD, including occult infection given the precedent established by HHV-8 infection.
View Article and Find Full Text PDFJAMA Netw Open
January 2025
Division of Neonatology, Department of Pediatrics, Willem-Alexander Children's Hospital, Leiden University Medical Center, Leiden, the Netherlands.
Importance: Preventive efforts in pregnancy-related alloimmunization have considerably decreased the prevalence of hemolytic disease of the fetus and newborn (HDFN). International studies are therefore essential to obtain a deeper understanding of the postnatal management and outcomes of HDFN. Taken together with numerous treatment options, large practice variations among centers may exist.
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