Immunoadsorption therapy for paraneoplastic syndromes

Paraneoplastic neurologic syndromes associated with systemic cancer are bei ng increasingly recognized. Although these syndromes are thought to be immu nologically mediated treatment with steroids, immunoglobulin and plasmaphar esis has been disappointing. Based on our preliminary experience with the t reatment of 6 cases of paraneoplastic neurologic syndromes with protein A i mmunoadsorption, an institutional, open-arm treatment protocol was establis hed. Since our original report we have treated an additional 7 patients wit h this method. The 13 cases were accrued over a 2 year period and included 10 women and 3 men with an average age of 63. The paraneoplastic syndromes included 6 cases of cerebellar degeneration, 3 cases of opsoclonus/myoclonu s, 3 cases of encephalomyelitis and 1 case of Lambert Eaten myasthenic synd rome. Primary cancers included 4 cases of small cell lung cancer, 2 cases o f breast cancer, 2 cases of lymphoma and 1 each of acinic cell cancer, chol angiocarcinoma, Merkel cell cancer, pancreatic adenocarcinoma and rectal ca ncer. Anti-neuronal antibody status, cerebrospinal fluid and neuroimaging s tudies as well as cancer staging and treatment protocols were reviewed. Neu rologic syndromes were clinically separated into component symptoms and sig ns for assessment of treatment effect. The treatment goal was a total of 6 sessions of protein A immunoadsorption given twice weekly. Twelve of 13 pat ients completed therapy and one patient developed cutaneous vasculitis duri ng the second session with termination of treatment. Of the remaining patie nts 3/12 had a complete response of the primary clinical symptom/sign while 6/12 had a partial response for a total response rate of 9/12 (75%). Toxic ity was limited to cutaneous vasculitis in 1 patient and an episode of hemi sensory changes in another patient. Current treatment of paraneoplastic neu rologic syndromes remains unsatisfactory. Despite the small number of patie nts in this report, protein A immunoadsorption is a promising therapy which deserves further study in a larger population of patients with paraneoplas tic syndromes.