Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature.
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.
Department of Pathology, Hôpital Necker-Enfants-Malades (AP-HP), Université Paris-Descartes, Paris, France.
T
T
Duong
F
F
Grange
N
N
Auffret
S
S
Aractingi
C
C
Bodemer
N
N
Brousse
O
O
Hermine
S
S
Fraitag
D018951:Antigens, CD20 / Q000276:immunology; D000951:Antigens, Neoplasm / Q000276:immunology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017252:CD3 Complex / Q000276:immunology; D018956:CD5 Antigens / Q000276:immunology; D002472:Cell Transformation, Viral / Q000276:immunology; D020031:Epstein-Barr Virus Infections / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D017730:Ki-1 Antigen / Q000276:immunology; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000276:immunology* / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012878:Skin Neoplasms / Q000276:immunology* / Q000473:pathology* / Q000821:virology; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
D018951:Antigens, CD20; D000951:Antigens, Neoplasm; D017252:CD3 Complex; D018956:CD5 Antigens; D017730:Ki-1 Antigen; D014740:Vidarabine; C024352:fludarabine