Refractory CIDP with chronic lymphocytic leukaemia responding to chemoimmunotherapy

Pract Neurol. 2020 Dec 29:practneurol-2020-002672. doi: 10.1136/practneurol-2020-002672. Online ahead of print.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) has a variable clinical course with 15% remaining refractory to treatment. We report a woman with severe refractory CIDP and coexisting chronic lymphocytic leukaemia (CLL) who improved dramatically after chemoimmunotherapy appropriate for the CLL, including rituximab. A subsequent CIDP relapse after 15 months responded again to similar treatment, and the improvement has been maintained with 3-monthly rituximab infusions as sole ongoing therapy. The case suggests that CIDP refractory to conventional treatment may have associated pathology, in this case haematological malignancy, and that treating the malignancy can effectively treat the CIDP.

Keywords: haematology; neuroimmunology; neurophysiology.