Richter's Syndrome Presenting as Primary Central Nervous System Lymphoma: Transformation of an Identical Clone

Bayliss, Katherine M.; Kueck, Brian; Hanson, Curtis A.; Matthaeus, William G.; Almagro, Urias A.

doi:10.1093/ajcp/93.1.117

Cited by 37 publications

(10 citation statements)

References 26 publications

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“…24,25 In chronic lymphocytic leukemia, such transformation is known as Richter syndrome and occurs in about 5% of patients. 25 The presenting sites of large-cell transformation in these patients are usually hematologic tissues, such as lymph nodes and bone marrow, and rarely occur in other sites, such as the gastrointestinal tract, 26 CNS, [27][28][29] and skin. 30 Ocular involvement by Richter syndrome is rare, with only 1 case reported in the literature.…”

Section: 20mentioning

confidence: 99%

Vitreoretinal Presentation of Secondary Large B-Cell Lymphoma in Patients With Systemic Lymphoma

et al. 2013

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Intraocular lymphomas represent a diverse group of hematologic malignant neoplasms involving different tissues within the eye. Predominant involvement of the retina and vitreous without uveal infiltration in systemic lymphoma, mimicking a primary vitreoretinal lymphoma, is extremely rare. Our study emphasizes the importance of systemic evaluation in addition to central nervous system evaluation in all patients with newly diagnosed vitreoretinal lymphoma.OBJECTIVE To determine the incidence of secondary intraocular lymphoma presenting as vitreoretinal infiltrates without central nervous system involvement, mimicking primary vitreoretinal lymphoma in patients with systemic lymphoma.DESIGN Retrospective review of all vitreous aspiration biopsy samples acquired because malignant neoplasm was suspected clinically that were obtained at our institution from January 1, 2000, through December 31, 2010. Review included patient clinical history, radiographic study findings, cytologic and/or histologic preparations, immunophenotypic study findings, treatment, and outcomes. SETTING Mayo Clinic pathology database.PARTICIPANTS Fifty-five patients with vitreous specimens available for review.EXPOSURES Vitreous aspiration biopsy. MAIN OUTCOME AND MEASURE Confirmation of the diagnosis of diffuse large B-cell lymphoma (DLBCL).RESULTS Of the 55 patients with vitreous specimens available for review, 3 (5%) had a DLBCL infiltration in the vitreous without any central nervous system involvement as a manifestation of systemic lymphoma. All 3 patients were men, aged 54, 66, and 73 years, and had blurred vision and floaters for several weeks before undergoing diagnostic vitrectomy. Ophthalmic examinations revealed clumps of vitreous cells but no choroidal involvement. One patient had no history of lymphoma; the diagnosis of vitreoretinal lymphoma was followed by DLBCL after a lymph node biopsy. The other 2 patients had low-grade B-cell lymphoma and chronic lymphocytic leukemia for 29 and 7 months before large-cell transformation in the eye. Patients were treated with systemic chemotherapy plus intraocular injections of rituximab or methotrexate sodium. CONCLUSIONS AND RELEVANCEVitreoretinal symptoms of DLBCL in patients with systemic lymphoma may be more frequent than previously thought (5% in our study). Not all lymphomas with vitreoretinal involvement represent primary intraocular lymphomas; thorough ophthalmologic evaluation in patients with visual symptoms and complete staging in patients with documented ocular lymphoma are of utmost importance.

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Section: 20mentioning

confidence: 99%

Vitreoretinal Presentation of Secondary Large B-Cell Lymphoma in Patients With Systemic Lymphoma

et al. 2013

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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%

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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“…Rarely, RS may present with extranodal involvement 23, 25, 106, 116–123. Extranodal sites of involvement include the gastrointestinal (GI) tract,23, 25, 106, 114, 116–120, 124 CNS,9, 105, 122, 123, 125 skin,121, 123, 126 eye,127 testis,128 and lung or kidney 114. Most patients with extranodal RS in the GI tract represent a true secondary neoplasm, but less frequently, a clonal relation between CLL and GI RS has been shown by clonality studies 25, 116…”

Section: Symptoms and Signs: Natural Historymentioning

confidence: 99%

Richter syndrome

Tsimberidou

Keating

2005

Cancer

248

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Richter's Syndrome Presenting as Primary Central Nervous System Lymphoma: Transformation of an Identical Clone

Cited by 37 publications

References 26 publications

Vitreoretinal Presentation of Secondary Large B-Cell Lymphoma in Patients With Systemic Lymphoma

Vitreoretinal Presentation of Secondary Large B-Cell Lymphoma in Patients With Systemic Lymphoma

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

Richter syndrome

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