title: Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone.
abstract: In Schnitzler syndrome, which is mostly diagnosed with a low and asymptomatic monoclonal peak, anakinra has always exhibited a complete but only transient control of the auto-inflammatory signs, which are induced by interleukin (IL)-1 auto-activation. We focused on the treatment of a case of Schnitzler syndrome with moderate macroglobulinemia peak. Anakinra failed to improve the severe inflammatory anaemia and the dysglobulinemia, but rituximab-dexamethasone-cyclophosphamide chemotherapy alone allowed a complete response. The correlation between the clinical, pro-inflammatory cytokines and dysglobulinemia complete controls with chemotherapy proves the following: (1) the dual action of this treatment in both the auto-inflammatory and dysglobulinemia components of the syndrome and (2) a different but entangled cytokine network in the pathogenesis of the auto-inflammatory and dysglobulinemia components of the syndrome.
authors.affiliation: Department of Clinical Immunology and Internal Medicine, Hôpital Antoine Béclère, AP-HP, Univerisité Paris 11, Clamart, France.
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Claire
authors.initials: C
authors.lastname: Pressiat
authors.forename: Maria
authors.initials: M
authors.lastname: Pricopi
authors.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.forename: François
authors.initials: F
authors.lastname: Boue
authors.forename: Maria-Angela
authors.initials: MA
authors.lastname: Lievre-Castilla
authors.forename: Anne
authors.initials: A
authors.lastname: Marfaing-Koka
authors.forename: Sophie
authors.initials: S
authors.lastname: Prevot
authors.forename: Audrey
authors.initials: A
authors.lastname: Decottignies
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D008875:Middle Aged; D012074:Remission Induction; D000069283:Rituximab; D019873:Schnitzler Syndrome / Q000188:drug therapy*; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D053590:Interleukin 1 Receptor Antagonist Protein; D000069283:Rituximab; D003907:Dexamethasone; D003520:Cyclophosphamide