A 22-year-old woman was referred to our hospital for further examination of an incidentally discovered hypervascular pelvic tumor with a maximum diameter of 10 cm. Although Castleman disease was suspected based on the imaging findings and pathologic findings of the needle biopsy, a definitive diagnosis was not made. Preoperative transcatheter arter...
Castleman disease (CD) is a rare lymphoproliferative disorder known to represent at least four distinct clinicopathologic subtypes. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on number of enlarged lymph nodes (unicentric versus multicentric), according to...
Published in Insights into Imaging
Castleman disease (CD) describes a group of rare hematologic conditions involving lymphadenopathy with characteristic histopathology and a spectrum of clinical abnormalities. CD is divided into localized or unicentric CD (UCD) and multicentric CD (MCD) by imaging. MCD is further divided based on etiological driver into human herpesvirus-8-associate...
Published in Rheumatology (Oxford, England)
While the aetiology of idiopathic multicentric Castleman disease (iMCD) remains unclear, the involvement of autoinflammatory mechanisms has been suggested. Herein we report a Japanese patient with iMCD with a novel heterozygous Ile729Met mutation in exon 10 of the Mediterranean fever (MEFV) gene. We performed genetic analysis via targeted next-gene...
Published in Cell Reports Medicine
Pierson et al. describe the feasibility of a patient-powered natural history registry for studying Castleman disease. They pair a traditional registry with a patient-powered approach, in which patients self-enroll and data collection is centralized. Clinical insights support treatment guidelines, and de-identified linkage to a biobank enables trans...
Published in Urology Case Reports
Renal Cell Carcinoma (RCC) corresponds to 3% of the neoplasms in the adults. Surgery is the main mode of treatment, which can be associated toretroperitoneal lymphadenectomy in the presence of clinically tumor positive lymph nodes. Castleman Disease (CD) is a rare lymphoproliferative disorder, with little-known etiopathogenesis. It rarely affects t...
Published in BMC Nephrology
BackgroundTAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis (or renal dysfunction), and organomegaly. TAFRO syndrome has only recently been described, and many clinicians are unaware of this disease, leading to delays in diagno...
Published in Journal of Laboratory Physicians
Castleman disease (CD), or angiofollicular hyperplasia, or giant lymph node hyperplasia, is a heterogeneous benign lymphoproliferative disorder of unknown etiology. It has three distinct histologic subtypes (hyaline vascular, plasma cell, and mixed hyaline vascular plasma cell types) as well as unicentric Castleman disease (UCD) and multicentric Ca...
Published in Autoimmunity Highlights
IntroductionMarfan syndrome, G6PD deficiency, systemic lupus erythematosus (SLE), and Castleman disease are four distinctive, thoroughly investigated entities whose coincidence was never reported. However, occurrence in pairs was sporadically mentioned in literature.Case presentationWe report a 15-year-old Caucasian G6PD deficient Marfan male patie...
Published in Dermatologic therapy
Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with an underlying malignancy. The survival rate at 5 years is reported to be as low as 38%. Thalidomide is a medication with strong anti-inflammatory, immunomodulatory, antiangiogenic, and sedative properties. Recently, the successful application of thalidomide in sever...
