Long-term Cytapheresis in the Treatment of Sézary-syndrome

Abstract

Lymphocytaphereses using the Fenwal CS 3000 cell separator were performed in three patients with far advanced disease of Sézary-syndrome that was refractory to standard form of therapy. Patients underwent cytapheresis two to three times per week until the WBCs counts were regressed. Thereafter, one pheresis per week was performed for a total of 29 (Pat. A), 18 (Pat. B) to 70 (Pat. C) procedures during periods of 13 (A), 4 (B) to 19 (C) months.

During each pheresis the volume of blood processed was 6-7000 mls. The cytaphereses resulted in an average WBCs reduction of 25 to 35% and of 35-56% of the original peripheral blood Sézary-cell counts. Lymphocytes counts dropped by 47 ± 12% and 87 to 95% of the collected cells were lymphocytes. In addition to the fall in absolute numbers of circulating lymphocytes a change in the relative portions of lymphocytes subpopulations was observed. T-cells were reduced and concurrently B-cells increased. Significant side effects did not occured during or following cytaphereses. Long-term pheresis can effectively treat symptmoms also in older patients resulting in preferential loss of T-cells and Sézary-cells without exposing the patients to dangerously large shifts in fluid volume. The results suggest also that there may be selected patients for whom lymphocytapheresis is an useful therapy, but no in vitro test can reliably separate responders from non-responders, and some patients become refractory to further cytapheresis-therapy after initial clinical remission. While cytapheresis does not appear to be sufficient as a sole therapeutic modality in this disease, it may be for some patients, if initiated early in the course of the disorder, result in clinical improvement. It may also be safely used even when the peripheral WBCs count is normal and for some patients there appears to be additive benefit of the procedure.