Efficacy of the adapted protocol of complex immunosuppressive therapy regimen "fludarabine-cyclophosphamide-dexamethasone- rituximab" in CLIPPERS syndromee (clinical case)

Abstract

CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) syndrome is a chronic lymphocytic inflammation of the pons with perivascular accumulation of contrast agent responding to glucocorticosteroid therapy (GCS). This immune- mediated brainstem lesion syndrome requires extensive differential diagnosis. The literature provides descriptions of CLIPPERS with delayed response to GCS or refractory to GCS. Such cases require a customized treatment tactics. In the clinical case of CLIPPERS presented by the authors, it was possible to achieve disease remission using an adapted chemotherapeutic protocol regimen "fludarabine-cyclophosphamide- dexamethasone-rituximab". During the follow-up, the patient did not have any disease relapses. Persistent immunosuppressive effect a year after therapy according to flow cytometry indicated effective inhibition of proinflammatory phenotypes of cellular components. The stabilization of the condition with the improvement of parameters according to the rating scales was noted. The study of cerebrospinal fluid 12 months after nonselective immune depletion indicated a decrease in the myelin basic protein content, normalization of lambda free light chains, the blood-brain barrier permeability and a change in the synthesis type from oligoclonal to polyclonal. The authors conclude that using this method, a long-term clinical, radiological and immunological remission of potentially any autoimmune process can be achieved. KEYWORDS: CLIPPERS, immunosuppressive therapy, fludarabine, rituximab, cyclophosphamide, immunosuppression, autoimmune disease. FOR CITATION: Polushin A.Yu., Zalyalov Yu.R., Tsynchenko A.A. et al. Efficacy of the adapted protocol of complex immunosuppressive therapy regimen "fludarabine-cyclophosphamide-dexamethasone-rituximab" in CLIPPERS syndrome (clinical case). Russian Medical Inquiry. 2022;6(10):580–588 (in Russ.). DOI: 10.32364/2587-6821-2022-6-10-580-588.