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Richter's syndrome of the brain and the spinal cord
  1. T D Graves1,
  2. G P Collins2,
  3. A Parry1
  1. 1Department of Neurology, Oxford Radcliffe Hospitals NHS Trust, John Radcliffe Hospital & Churchill Hospital, Oxford, UK
  2. 2Department of Haematology, Oxford Radcliffe Hospitals NHS Trust, John Radcliffe Hospital & Churchill Hospital, Oxford, UK
  1. Correspondence to Dr Allyson Parry, Department of Neurology, John Radclifffe Hospital, 3rd Floor, West Wing, Headley Way, Oxford OX3 9DU, UK; allyson.parry{at}orh.nhs.uk

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Case history

A man in his late 60s was found to have lymphadenopathy and splenomegaly on routine CT. This progressed for 6 months, and a lymph node biopsy revealed monomorphic small B lymphocytes. Bone marrow aspirate showed minimal (5%) infiltration, and immunophenotyping confirmed a small population of clonal B cells. Stage IIIS B-cell chronic lymphocytic leukaemia (CLL) was diagnosed and a “watch and wait” policy adopted. He developed abdominal pain, anorexia, weight loss, diarrhoea, pruritus and night sweats and was given chlorambucil, to which he had a good response. Nine months later, he developed lymphadenopathy in the left groin, and a biopsy confirmed CLL. Second-line chemotherapy with fludarabine, cyclophosphamide and rituximab was started. As there was no response, high-dose methylprednisolone was given. There was no 17p deletion, and molecular analysis of the immunoglobulin locus was normal. CT of the abdomen 6 months later showed progression, and biopsy of the abdominal mass confirmed Richter's transformation (large B-cell non-Hodgkin lymphoma (NHL)). He was therefore given cyclophosphamide, doxorubicin, vincristine, prednisolone and rituximab, which lead to a clinical and radiological response.

Neurological symptoms developed after the fourth cycle of cyclophosphamide, doxorubicin, vincristine, prednisolone and rituximab with 2 weeks of ascending numbness and 2 days of unsteadiness with increasing fatigue. On examination, tone and power were normal. Reflexes were difficult to elicit, …

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