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Unicentric Castleman disease information


Unicentric Castleman disease
Other namesGiant lymph node hyperplasia, lymphoid hamartoma, angiofollicular lymph node hyperplasia
Micrograph of lymph node biopsy demonstrating hyaline vascular features consistent with Castleman disease
SpecialtyHematology, immunology, rheumatology, pathology
Diagnostic methodBased on patient history, physical exam, laboratory testing, medical imaging, histopathology
FrequencyApproximately 5000-6000 new cases per year in the United States

Unicentric Castleman disease is a subtype of Castleman disease (also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia), a group of lymphoproliferative disorders characterized by lymph node enlargement, characteristic features on microscopic analysis of enlarged lymph node tissue, and a range of symptoms and clinical findings.

People with unicentric Castleman disease (UCD) have an enlarged lymph node or multiple enlarged lymph nodes in a single lymph node region. It is the most common subtype of Castleman disease, symptoms are typically mild, abnormalities on blood tests are uncommon, organ dysfunction is uncommon, and surgical treatment is curative in the majority of patients. The cause of UCD is not known.

Castleman disease is named after Benjamin Castleman, who first described the disease in 1956. The Castleman Disease Collaborative Network is the largest organization focused on the disease and is involved in research, awareness, and patient support.

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