| Idiopathic multicentric Castleman disease | |
|---|---|
| Other names | Giant lymph node hyperplasia, lymphoid hamartoma, angiofollicular lymph node hyperplasia |
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| Micrograph of lymph node biopsy demonstrating hyaline vascular features consistent with Castleman disease | |
| Specialty | Hematology, immunology, rheumatology, pathology |
| Diagnostic method | Based on patient history, physical exam, laboratory testing, medical imaging, histopathology |
| Frequency | approximately 1500-1800 new cases per year in the United States |
Idiopathic multicentric Castleman disease (iMCD) 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 iMCD have enlarged lymph nodes in multiple regions and often have flu-like symptoms, abnormal findings on blood tests, and dysfunction of vital organs, such as the liver, kidneys, and bone marrow.
iMCD has features often found in autoimmune diseases and cancers, but the underlying disease mechanism is unknown. Treatment for iMCD may involve the use of a variety of medications, including immunosuppressants and chemotherapy.
Castleman disease was named after Dr. 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.