Schnitzler syndrome complete resolution of symptoms on treatment with anakinra after 12 years of unsuccessful therapy with other regimens

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Authors

ADAM Zdeněk POUR Luděk KREJČÍ Marta NEUBAUER Jiří PRÁŠEK Jiří BÜCHLER Tomáš HÁJEK Roman

Year of publication 2009
Type Article in Periodical
Magazine / Source Cancer Therapy
MU Faculty or unit

Faculty of Medicine

Citation
Field Oncology and hematology
Keywords Schnitzler Syndrome; Gammapathy;Urticaria; Anakinra
Description Schnitzler syndrome is a rare disorder characterized by chronic urticaria, the presence of monoclonal class IgM immunoglobulin, lymphadenopathy and fevers. We report on a patient who was diagnosed in 1996 and over the next 12 years underwent a number of therapies. Bone pain associated with proven osteolytic and osteosclerotic lesions resolved on regular bisphosphonate treatment with pamidronate and, later, clodronate. The patient had transient benefit from treatments with interferon-alpha and with PUVA. No therapeutic effects were seen with high-dose dexamethasone, 2-chlordeoxyadenosin, cyclosporine, or the combination of bortezomib, thalidomide, and dexamethasone. However, on therapy with anakinra the patients skin symptoms resolved completely and the values of CRP and hemoglobin normalized.
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