Isolated Richter's syndrome in central nervous system: case report

Resende, Lucilene Silva Ruiz e; Bacchi, Carlos E.; Resende, Luiz Antônio de Lima; Gabarra, Roberto Colichio; Niéro-Melo, Lígia

doi:10.1590/s0004-282x2005000300031

Cited by 8 publications

(5 citation statements)

References 5 publications

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“…By the year 2005, 13 cases of extranodal central nervous system involvement had been reported (Robertson et al , 1993; Resende et al , 2005; `O’Neill et al , 1989;Lane et al , 1988; Bayliss et al , 1990; Mahé et al , 1994; Gilles et al , 1998; Agard et al , 1999). Five of them had isolated leptomeningeal involvement (Robertson et al , 1993; Lane et al , 1988; Agard et al , 1999), while the other eight had parenchymal involvement.…”

Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

“…Five of them had isolated leptomeningeal involvement (Robertson et al , 1993; Lane et al , 1988; Agard et al , 1999), while the other eight had parenchymal involvement. The malignant characteristics of large cell lymphoma may result in neuroradiological features similar to multiform glioblastoma (Resende et al , 2005). Lymphoma of the cerebral parenchyma has a very poor prognosis resulting in early death despite treatment (Resende et al , 2005).…”

Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

“…The malignant characteristics of large cell lymphoma may result in neuroradiological features similar to multiform glioblastoma (Resende et al , 2005). Lymphoma of the cerebral parenchyma has a very poor prognosis resulting in early death despite treatment (Resende et al , 2005). Isolated brain Richter syndrome without evidence of systemic lymphoma is even rarer, and only seven cases have been reported to date (Bagic et al , 2007).…”

Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

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Richter syndrome: a review of clinical, ocular, neurological and other manifestations

Omoti

2008

Br J Haematol

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Summary Richter syndrome describes the development of high‐grade non‐Hodgkin lymphoma (NHL) or Hodgkin lymphoma in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). Richter transformation occurs in 3·3 to 10·6% of patients with CLL. The large cell lymphoma clone occurs by transformation of the original CLL clone in the majority of patients, and as a separate and independent neoplasm in fewer cases. Richter transformation may be triggered by viral infections, such as Epstein‐Barr virus infection, which are common in immunosuppressed patients. Trisomy 12 and chromosome 11 abnormalities, as well as multiple genetic defects, have been described in patients with Richter syndrome. These abnormalities may cause CLL cells to proliferate and, by facilitating the acquisition of new genetic abnormalities, to transform into Richter syndrome cells. Presenting features typically include a rapid clinical deterioration with fever in the absence of infection, progressive lymph node enlargement, and an elevation in serum lactate dehydrogenase. Extranodal Richter syndrome has also been reported to occur in the central nervous system, eye, gastrointestinal system, nose, skin, face, bone and bronchus. The therapeutic options include cytoreductive therapy consisting of chemotherapy and immunotherapy, followed by allogeneic stem cell transplantation as postremission therapy.

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Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

Section: Ocular Neurological and Other Extranodal Manifestationsmentioning

confidence: 99%

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Richter syndrome: a review of clinical, ocular, neurological and other manifestations

Omoti

2008

Br J Haematol

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“…In contrast, it is estimated that up to 5% of patients with primary NHL have CNS involvement 4. CNS disease usually occurs in the context of systemic Richter's syndrome and presents as tumour or malignant meningitis, but isolated CNS disease has been described5 (see supplementary table). Treatment is cytoreductive, based on NHL therapy, and increasingly includes rituximab.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, the development of a second malignancy due to cytotoxic medication would not account for this excess risk. Richter's syndrome may occur in the absence of systemic features and in patients whose CLL appears to be in remission5; therefore, a high index of clinical suspicion is required to make the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Richter's syndrome of the brain and the spinal cord

Graves

Collins

Parry³

2010

Journal of Neurology, Neurosurgery & Psychiatry

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Isolated Richter’s transformation of the brain

et al. 2007

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Patients with B-cell chronic lymphocytic leukemia (CLL) have an increased risk of second malignancy and may develop diffuse large-cell non-Hodgkin's lymphoma (DLCL) also known as Richter's syndrome (RS). Only seven cases of isolated brain RS without evidence of systemic lymphoma have been reported to date. We describe a case of isolated DLCL of the brain in a 58-year-old female patient with a 3 year history of B-cell CLL. The patient presented with falls due to left leg paresis and showed non-specific neuroimaging findings. Brain biopsy confirmed the diagnosis of DLCL and CLL restaging failed to demonstrate evidence of RS outside the CNS. The patient was treated with whole brain radiation therapy and was discharged 4 weeks later on weekly rituximab infusions. This report extends the previous experience by providing a detailed clinical, neuroradiological and pathological description of isolated RS of the brain. Early identification and timely CNS directed treatment may alter morbidity and positively influence quality of life.

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Isolated Richter's syndrome in central nervous system: case report

Cited by 8 publications

References 5 publications

Richter syndrome: a review of clinical, ocular, neurological and other manifestations

Richter syndrome: a review of clinical, ocular, neurological and other manifestations

Richter's syndrome of the brain and the spinal cord

Isolated Richter’s transformation of the brain

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