Castlemans Disease, Affiliation: Department of Radiology, Cork University Hospital, Cork, Ireland. Open
Castleman's disease (Angio-follicular lymph node hyperplasia, lymphoid hamartoma)
Contents
Definition
Castleman's disease is a benign, rare, disease that includes lymph nodes with different localities, most commonly in the mediastinum (thorax). Also reported on the neck. Described by Benjamin Castleman in 1956.
Symptoms
Two types:
-
Uni-centric
- Only one lymph node is attacked
- Debuts with a large, well-defined lymph node without special pain
- Rarely changes in blood samples
-
Multi-Centric
- Several enlarged lymph nodes, of which 50% is associated with Kaposi's sarcoma herpesvirus (HHV-8), NB HIV infection
- B symptoms: Fever, anemia, weight loss
Castleman's disease with kidney filling. Saed-Abdul-Rahman J. CC BY NC 3.0
Diagnosis
Biopsy (may be difficult to interpret)
Incorrect diagnosis (similar conditions, differential diagnoses)
- POEMS syndrome (Polynevropy, Oragano megali, Endocrinopathy, Monoclonal gammopathy, Skin symptoms)
- IgG4 related disease
- Histiocytosis (Rosai Dorfman Disease and Others)
- Reactive lymph node tumor
- Lymphoma (Hodgkin og non-Hodgkin)
- Kaposis sarcoma (by HIV)
- Metastases
Treatment
- Unicentric Type: Surgical removal at 90% is successful without relapse
- Multisentric Type: Antiviral Treatment. Immunosuppressive drugs
- Siltuximab (IL-6 inhibitor) showing promising results (Lancet 2014)
Literature
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