Article Text

Download PDFPDF
Letter
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

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

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, …

View Full Text

Footnotes

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.