Castleman's disease
| Castleman disease | |
|---|---|
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| Micrograph of Castleman disease, hyaline vascular variant, exhibiting the characteristically expanded mantle zone and a radially penetrating sclerotic blood vessel ("lollipop" sign). H&E stain. | |
| Classification and external resources | |
| ICD-10 | D47.4 |
| ICD-9-CM | 785.6 |
| DiseasesDB | 2165 |
| MeSH | D005871 |
Castleman disease, also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia, is a group of uncommon lymphoproliferative disorders that share common lymph node histological features. It is named after Benjamin Castleman.
Castleman's disease has two main forms: It may be localized to a single lymph node (unicentric) or occur systemically (multicentric).
The unicentric form can usually be cured by surgically removing the lymph node, with a 10-year survival of 95%.
Multicentric Castleman disease (MCD) involves hyperactivation of the immune system, excessive release of proinflammatory chemicals (cytokines), proliferation of immune cells (B cells and T cells), and multiple organ system dysfunction. Castleman disease must be distinguished from other disorders that can demonstrate "Castleman-like" lymph node features, including reactive lymph node hyperplasia, autoimmune disorders, and malignancies. Multicentric Castleman's disease is associated with lymphoma and Kaposi's sarcoma.
There are three sub-types of Castleman disease.
Unicentric Castleman disease involves "Castleman-like" lymph node changes at only a single site. It usually has few or no symptoms other than those directly associated with the enlargement of the lymph node. In at least 90% of cases, removal of the enlarged node is curative with no further complications.
Multicentric Castleman disease (MCD) involves "Castleman-like" lymph node changes at multiple sites and patients often demonstrate intense episodes of systemic inflammatory symptoms, polyclonal/reactive lymphocyte and plasma cell proliferation, autoimmune manifestations, and multiple organ system impairment.
Lymph node abnormalities and organ dysfunction in Castleman disease are caused by hypersecretion of cytokines. IL-6 is the most commonly elevated cytokine, but some patients may have normal IL-6 levels and present with non-iron-deficient microcytic anemia.
The release of these cytokines is caused by HHV-8 in HHV-8-associated MCD. The cause of the release of cytokines in idiopathic MCD has been hypothesized to be caused by either a somatic mutation, a germline genetic mutation, or a non-HHV-8-virus.
A new model of pathogenesis (lymph node changes are not “benign tumors” that secrete cytokines, but reactive changes due to excessive cytokine release from an as-yet unknown cause) and a new classification system for MCD (based on HHV-8 status) have ensued. CDCN has launched a platform for online discussion among physicians and researchers, developed a global research agenda, and launched a global patient community in partnership with EURODIS and NORD. Current strategic priorities include: 1) establishing a global patient registry, 2) empowering the global patient community to support one another and join the fight against CD, and 3) distributing high-impact research grants.
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