Demyelinating brain lesions developing in a patient with chronic lymphocytic leukaemia shortly after treatment with a fludarabine containing regimen

Autoimmune manifestations are known to occur in patients with chronic lymphocytic leukaemia (CLL) and of these haemolytic anaemia and immune thrombocytopenia are the most well recognized. Autoimmunity may also be triggered by some of the therapeutic agents used like purine analogues and these events may sometimes be severe and even fatal. Non- haematological autoimmune stigmata occur far less frequently and are rarely encountered.Here we report a 59 year-old-woman, with CLL, who complained of recurrent headache starting one month after completing 6 cycles of FCR combination therapy (fludarabine, cyclophosphamide and rituximab). CT scan of the brain showed a contrast enhancing lesion of 1 cm in diameter, with surrounding oedema in the right frontal lobe. Brain MRI revealed ring enhancing lesions in the right frontal lobe and some additional small lesions in the left parietal lobe. Brain biopsy showed an inflammatory demyelinating lesion, not associated with JC virus. The patient subsequently improved after steroid therapy. Currently, after 2 years of follow-up, she remains in complete hematologic remission, has no neurological deficits and is carefully followed by a team of neurologists and haematologists.Treating physicians should be aware of this rare autoimmune inflammatory demyelinating lesion which can occur in patients with CLL during the course of treatment and that may be linked to treatment with purine analogues like fludarabine. This article is protected by copyright. All rights reserved.

Keywords: Autoimmune disorders; CLL; brain lesions; chronic lymphocytic leukaemia; demyelination; fludarabine; therapy.