Centroblastic transformation of chronic lymphocytic leukaemia with primary skin involvement-cutaneous presentation of Richter's syndrome

Abstract

The development of a large-cell non-Hodgkin's lymphoma in a patient suffering from chronic lymphocytic leukaemia is known as Richter's syndrome, representing one of the possible anaplastic transformations of the leukaemia. Cutaneous involvement is an extremely rare event. The case of a 45-year-old man with B-cell chronic lymphocytic leukaemia is reported. Five years later multiple cutaneous nodules developed on his extremities. Biopsies of the skin showed a diffuse large-cell lymphoma, bearing the same cell surface immunoglobulin light chain isotype as the leukaemia, suggesting the original relationship between both neoplasms.

Cutaneous Richter syndrome: Report of 3 cases from one institution

“…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.…”

“…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.…”

Cutaneous Richter syndrome: a better place to transform?

“…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 .…”

“…Analysis of immunoglobulin gene rearrangements indicates that in 70–80% of cases, CLL and RS arise from the same B‐cell clone. However, in other cases the development of RS reflects the emergence of a separate and independent large B‐cell lymphoma distinct from the original CLL clone . The molecular mechanisms involved in transformation to RS are still poorly understood.…”

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

“…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).…”