Background: Castleman’s disease (CD) is a lymphoprolifera-tive disorder, probably of reactive origin but poorly regulated by a defective immune system, and therefore prone to oligoclonal evolution. Diagnosis is made by histopathological features, involvement of multiple lymph nodes, evidence of a multisystem involvement, and an idiopathic nature. The course is unpredictable, but a high mortality rate is reflected in the literature. Patients were treated with either corticosteroids or chemotherapy designed for lymphoma. Case Report: We present a case of a 63-year-old woman with multicentric CD who was admitted with weight loss, night sweat, fever, and generalized lymphadenopathy. Laboratory investigations showed a Coombs-positive hemolytic anemia and an elevated polyclonal immunglobulin level. Lymph node biopsy showed pulpic hyperplasia and proliferation of venules. Clinically, radiologically and biochemically, an autoimmune disorder, an infection as well as an underlying neoplasia were highly unlikely. Despite the introduction of immunosuppressive therapy with steroids (prednisone 100 mg/day), the patient developed a severe radiculopathy, predominantly with a paresis of the upper extremities in association with a total ophtalmoplegia and a dysphagia, consistent with the Miller-Fisher syndrome (ophtalmoplegia, ataxia, and areflexia). Therefore, plasma separation was performed for 6 days. In addition, cyclophosphamide was administered in a dosage of 3 g/m2. Following 2 courses of cyclophosphamide infusion a significant reduction in lymphadenopathy and an almost complete resolution of neurological symptoms was documented. After 6 courses chemotherapy with CHOP (cyclophosphamide, doxorubicine, dexamethasone and vincristin) the patient was still in partial remission. She died of pneumonia one year after diagnosis of Castleman’s disease. Conclusion: This is the first reported case of Castleman’s disease associated with polyradiculitis. Appropriate treatment for both, the polyradiculitis and the Castleman’s disease proved to be effective.

Keywords:
Castleman’s disease, Polyradiculitis, Plasmapheresis, · Cyclophosphamide
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© 1997 S. Karger AG, Basel
1997
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