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: Achille Aouba, Claire Pressiat, Maria Pricopi, Sophie Georgin-Lavialle, François Boue, Maria-Angela Lievre-Castilla, Anne Marfaing-Koka, Sophie Prevot, Audrey Decottignies
• Journal: Dermatology (Basel, Switzerland) (Dermatology) Vol. 230 Issue 1 Pg. 18-22 ( 2015) ISSN: 1421-9832 [Electronic] Switzerland
• PMID: 25471063 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2014 S. Karger AG, Basel.

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Interleukin 1 Receptor Antagonist Protein
• Rituximab
• Dexamethasone
• Cyclophosphamide

Topics

• Antibodies, Monoclonal, Murine-Derived (administration & dosage)
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Cyclophosphamide (administration & dosage)
• Dexamethasone (administration & dosage)
• Female
• Humans
• Interleukin 1 Receptor Antagonist Protein (therapeutic use)
• Middle Aged
• Remission Induction
• Rituximab
• Schnitzler Syndrome (drug therapy)
• Waldenstrom Macroglobulinemia (drug therapy)