A 63-YEAR-OLD PATIENT WITH MULTICENTRIC CASTLEMANS-DISEASE AND SEVEREPOLYRADICULITIS

Background: Castleman's disease (CD) is a lymphoproliferative disorder , probably of reactive origin but poorly regulated by a defective immu ne system, and therefore prone to oligo-clonal evolution. Diagnosis is made by histopathological features, involvement of multiple lymph nod es, evidence of a multisystem involvement, and an idiopathic nature. T he course is unpredictable, but a high mortality rate is reflected in the literature. Patients were treated with either corticosteroids or c hemotherapy designed for lymphoma. Case Report: We present a case of a 63-year-old woman with multicentric CD who was admitted with weight l oss? night sweat, fever, and generalized lymphadenopathy. Laboratory i nvestigations showed a Coombs-positive hemolytic anemia and an elevate d polyclonal immunglobulin level. Lymph node biopsy showed pulpic hype rplasia and proliferation of venules. Clinically, radiologically and b iochemically, an autoimmune disorder. an infection as well as an under lying neoplasia were highly unlikely Despite the introduction of immun osuppressive therapy with steroids (prednisone 100 mg/day), the patien t developed a severe radiculopathy, predominantly with a paresis of th e upper extremities in association with a total ophtalmoplegia and a d ysphagia, 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/m(2). Following 2 courses of cyclophosphamide infusion a signific ant reduction in lymphadenopathy and an almost complete resolution of neurological symptoms was documented. After 6 courses chemotherapy wit h 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 f irst reported case of Castleman's disease associated with polyradiculi tis. Appropriate treatment for both, the polyradiculitis and the Castl eman's disease proved to be effective.