Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Although the pathogenesis is not completely understood, the role of interleukin (IL)-6 in unicentric disease and the roles of IL-6 and human herpes virus-8 in multicentric disease are well defined. Unicentric disease is typically localized and symptoms are minimal and treated locally. Multicentric disease is systemic and clinically characterized by generalized lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. Systemic therapies are primarily given. Several malignant diseases including lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, and amyloidosis can be associated with Castleman disease. In this paper, recent information about Castleman disease, which is a rare disease, is summarized.
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http://dx.doi.org/10.4274/tjh.galenos.2021.2021.0440 | DOI Listing |
Int J Hematol
January 2025
Division of Hematology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.
The study aimed to investigate the therapeutic effect of various initial treatments incorporating glucocorticoid (GC) in TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly). Cases of TAFRO syndrome up to November 2023 were retrospectively collected. Overall survival (OS) and resistance to GC therapy were assessed, with resistance analyzed based on the time to the next treatment or death (TTNTD).
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of Rheumatology, US Department of Veterans Affairs, Fresno, California, USA.
Castleman's disease (CD), also called angiofollicular lymphoid hyperplasia, is a rare lymphoproliferative illness with two unique variants: unicentric disease and multicentric disease (MCD). The multicentric variant is rare and presents as a systemic illness with symptoms like peripheral lymphadenopathy, splenomegaly, anaemia and systemic inflammatory symptoms. Given the vague and systemic presentation, this variant can be difficult to differentiate from infection and other autoimmune diseases.
View Article and Find Full Text PDFInt J Surg Case Rep
January 2025
Department of Surgical Oncology, Shaukat Khanum Memorial Cancer Hospital & Research Center, Pakistan; Shaukat Khanum Memorial Cancer Hospital and Research Centre, 7A Block R-3, M.A.Johar Town, Lahore, Pakistan. Electronic address:
Introduction: Castleman disease is a rare lymphoproliferative disorder, subdivided into three types: unicentric Castleman disease, idiopathic multicentric Castleman disease and human herpesvirus-8 (HHV8) associated multicentric Castleman disease. The retroperitoneum comprises only 13 % of the cases.
Case Presentation: We report a case of a 36-year-old female who presented with skin lesions in a dermatology clinic.
Am J Hematol
January 2025
Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions.
View Article and Find Full Text PDFBMC Pulm Med
January 2025
Department of Pulmonary and Critical Care Medicine, School of Medicine, Zhongshan Hospital of Xiamen University, Xiamen University, Xiamen, Fujian, China.
Introduction: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is commonly used for diagnosing mediastinal lymphadenopathy. Despite a low complication rate, severe hemorrhage can occur which is reported in this literature, particularly in hypervascular conditions like Castleman disease.
Methods: A 54-year-old male with idiopathic multicentric Castleman disease underwent EBUS-TBNA for mediastinal lymph node sampling.
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