Micrograph of Castleman disease showing hyaline vascular features including atrophic germinal center, expanded mantle zone, and a radially penetrating sclerotic blood vessel ("lollipop" sign). H&E stain.
Specialty
Immunology, angiology
Symptoms
fever, unintended weight loss, fatigue, night sweats, nausea, enlarged liver or spleen.[1]
Castlemandisease (CD) describes a group of rare lymphoproliferative disorders that involve enlarged lymph nodes, and a broad range of inflammatory symptoms and laboratory abnormalities. Whether Castleman disease should be considered an autoimmune disease, cancer, or infectious disease is currently unknown.
Castleman disease includes at least three distinct subtypes: unicentric Castleman disease (UCD), human herpesvirus 8 associated multicentric Castleman disease (HHV-8-associated MCD), and idiopathic multicentric Castleman disease (iMCD). These are differentiated by the number and location of affected lymph nodes and the presence of human herpesvirus 8, a known causative agent in a portion of cases. Correctly classifying the Castleman disease subtype is important, as the three subtypes vary significantly in symptoms, clinical findings, disease mechanism, treatment approach, and prognosis. All forms involve overproduction of cytokines and other inflammatory proteins by the body's immune system as well as characteristic abnormal lymph node features that can be observed under the microscope. In the United States, approximately 4,300 to 5,200 new cases are diagnosed each year.[2]
Castleman disease is named after Benjamin Castleman, who first described the disease in 1956. The Castleman Disease Collaborative Network is the largest organization dedicated to accelerating research and treatment for Castleman disease as well as improving patient care.[3]
^"Castleman disease". mayoclinic.org. Mayo Clinic. Retrieved 5 June 2022.
^Yu, Li; Tu, Meifeng; Cortes, Jorge; Xu-Monette, Zijun Y.; Miranda, Roberto N.; Zhang, Jun; Orlowski, Robert Z.; Neelapu, Sattva; Boddu, Prajwal C.; Akosile, Mary A.; Uldrick, Thomas S. (2017-03-23). "Clinical and pathological characteristics of HIV- and HHV-8–negative Castleman disease". Blood. 129 (12): 1658–1668. doi:10.1182/blood-2016-11-748855. ISSN 0006-4971. PMC 5364343. PMID 28100459.
Idiopathic multicentric Castlemandisease (iMCD) is a subtype of Castlemandisease (also known as giant lymph node hyperplasia, lymphoid hamartoma, or...
Unicentric Castlemandisease is a subtype of Castlemandisease (also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph...
The CastlemanDisease Collaborative Network (CDCN) is an organization focused on research and awareness of Castlemandisease. It was founded in 2012 and...
University of Pennsylvania. He is most well known for his research into Castlemandisease. David C. Fajgenbaum was born and grew up in Raleigh, North Carolina...
as a heralding sign of multicentric Castlemandisease (MCD), multiple myeloma and other lymphoproliferative diseases. Cherry angiomas occur in all races...
R S T U V W X Y Z This list contains acronyms and initials related to diseases (infectious or non-infectious) and medical disorders. Acronyms in healthcare...
Epstein–Barr virus-associated lymphoproliferative diseasesCastlemandisease X-linked lymphoproliferative disease Lymphoproliferative disorders are a set of...
overlap with the signs and symptoms of multicentric Castlemandisease. The more common features of the disease are summarized in the acronym POEMS: Papilledema...
Lymphatic disease is a class of disorders which directly affect the components of the lymphatic system. Examples include Castleman'sdisease and lymphedema...
multicentric Castlemandisease (iMCD). Siltuximab has demonstrated significant efficacy and safety in patients with idiopathic multicentric Castlemandisease. Treatment...
cell lymphoma. Mantle zone expansion may be seen in benign, such as Castlemandisease, and malignancy, i.e., Mantle cell lymphoma. Tcl-1 is expressed in...
A viral disease (or viral infection) occurs when an organism's body is invaded by pathogenic viruses, and infectious virus particles (virions) attach...
following a biopsy in the mouth Kawasaki disease, Kikuchi-Fujimoto disease Rosai-Dorfman diseaseCastlemandisease Sarcoidosis Lupus erythematosus Cyclic...
philanthropist, and suffragist Benjamin Castleman (born 1906), American pathologist best known for Castleman'sdisease Boomer Castleman, American singer-songwriter...
against many of these diseases. The first such is tocilizumab, which has been approved for rheumatoid arthritis, Castleman'sdisease and systemic juvenile...
Caroli syndrome – Jacques Caroli Carrion disease – Daniel Alcides Carrión Castlemandisease – Benjamin Castleman Céstan–Chenais syndrome – Étienne Jacques...
arthritis. In Japan, tocilizumab is also approved for the treatment of Castleman'sdisease, a rare benign tumor of B cells. In May 2017, the FDA approved tocilizumab...
certain lymphoproliferative diseases such as autoimmune lymphoproliferative syndrome (ALPS), multicentric Castlemandisease, and post-transplant lymphoproliferative...
lymphoma, Hodgkin lymphoma, B-cell chronic lymphocytic leukemia, and Castlemandisease; they occur rarely in non-B cell hematological disorders such as myelodysplastic...
conjugate vaccine Porcine circovirus, a virus Plasma cell variant of Castlemandisease Penicillin V, a type of antibiotic Passenger Carrying Vehicle Propelling...
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