Richter's Syndrome Presenting as a Solitary Cutaneous Nodule

Novice, Fred M.; Mikhail, George R.; Maeda, Koichi; Hawley, Robert G.

doi:10.1111/j.1365-4362.1989.tb01307.x

Cited by 11 publications

(11 citation statements)

References 4 publications

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“…23 Cutaneous RS has only been described in a few cases in the literature. 2,[6][7][8][9][10][11][12][13][14] In this study, we describe a series of 3 cutaneous RS cases from a single institution with a long period of follow-up (up to 8 years). All 3 patients had histories of CLL.…”

Section: Discussionmentioning

confidence: 99%

“…5 Skin involvement of RS is rare with only a handful of cases reported in the literature. 2,[6][7][8][9][10][11][12][13][14] RS presenting as a skin lesion with the initial diagnosis made by skin biopsy specimen is quite rare. Histopathological examination of the affected organ shows an atypical lymphoid infiltrate composed of large cells with enlarged nuclei, prominent nucleoli, and abundant basophilic cytoplasm.…”

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confidence: 99%

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Cutaneous Richter syndrome: Report of 3 cases from one institution

Bandhlish

Fullen

et al. 2012

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Cutaneous Richter syndrome: Report of 3 cases from one institution

Bandhlish

Fullen

et al. 2012

Journal of the American Academy of Dermatology

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“…In a series of 39 patients with RS reported by Robertson et al ., cutaneous involvement was identified in two cases but they were not described in detail. Subsequently, there have been a further 10 reports describing 17 patients with cutaneous RS (Tables and ) . Of these 17 patients, disease stabilization following chemotherapy with or without radiotherapy was seen in five, with survival after treatment of up to 8 years in one case .…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Richter syndrome: a better place to transform?

et al. 2014

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The development of high-grade lymphoma in patients with chronic lymphocytic leukaemia is known as Richter syndrome (RS) and is associated with a grave prognosis, with a mean survival of 8 months despite treatment. Cutaneous RS has been described in a handful of cases and may be associated with a better outcome than the more common extracutaneous variants. We review the literature with particular emphasis on pathogenesis, treatment and survival of RS. We postulate that the absence of B symptoms and a normal lactate dehydrogenase level, presumably reflecting localized or limited disease, and a lower tumour burden, may explain the apparently better survival in some patients with cutaneous RS than with extracutaneous variants.

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“…It has been described in isolated case reports in which the mean age at onset was 71 years (40–82 years) [3,13,14,20,21,22,23]. The patients had either a rapidly growing tumor or multiple erythematoviolaceous nodules at various sites (extremities, nose, chest and limbs).…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

Duong¹,

Grange

Auffret

et al. 2010

Dermatology

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Background: Richter’s syndrome (RS) corresponds to the transformation of chronic lymphocytic leukemia (CLL) into high-grade lymphoma. RS can involve extranodal sites including the gastrointestinal tract, lungs and skin. Cutaneous RS is rare, we describe 4 cases with clinical manifestations, histological and immunohistological patterns, and outcome. Methods: Clinical data were analyzed and all patients’ skin biopsy samples stained with HE for the CD20, CD5, CD3 and CD30 antigens. Epstein-Barr-virus (EBV)-encoded early RNA and clonal rearrangements were also analyzed. Results: The patients’ mean age at CLL diagnosis was 57 years (53–62 years), with a male/female sex ratio of 3:1. The transformation to cutaneous RS occurred between 8 and 75 months after initial diagnosis and progressed to a fatal systemic disease in 3 cases, between 24 and 129 months. Cutaneous CLL was associated with earlier transformation in our series and could not be distinguished from RS on clinical grounds alone. All patients had a large-cell infiltrate and clonal rearrangements. Conclusions: The precise mechanism of RS is unclear, but a role of EBV has been suggested in fludarabine-treated CLL. For all our patients, the diagnosis of transformation was made on the basis of cutaneous localizations and led to intensified CLL treatment.

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Richter's Syndrome Presenting as a Solitary Cutaneous Nodule

Cited by 11 publications

References 4 publications

Cutaneous Richter syndrome: Report of 3 cases from one institution

Cutaneous Richter syndrome: Report of 3 cases from one institution

Cutaneous Richter syndrome: a better place to transform?

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

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