title: Primary digestive Richter's syndrome.
abstract: The clinical and morphologic transformation of 3 to 5% of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) is commonly referred to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clonal population. Clinical features in six patients with digestive Richter's syndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n = 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n = 4). Histological examination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n = 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one case. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than nodal Richter's syndrome. Indeed, appropriate surgical resection combined with chemotherapy led to partial or complete remission in four patients.
authors.affiliation: Department of Pathology, Hôpital du Haut-Lévêque, Pessac, France. marie.parrens@chu-bordeaux.fr
authors.forename: M
authors.initials: M
authors.lastname: Parrens
authors.forename: B
authors.initials: B
authors.lastname: Sawan
authors.forename: P
authors.initials: P
authors.lastname: Dubus
authors.forename: F
authors.initials: F
authors.lastname: Lacombe
authors.forename: G
authors.initials: G
authors.lastname: Marit
authors.forename: B
authors.initials: B
authors.lastname: Vergier
authors.forename: J
authors.initials: J
authors.lastname: Reiffers
authors.forename: A
authors.initials: A
authors.lastname: de Mascarel
authors.forename: J P
authors.initials: JP
authors.lastname: Merlio
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000951:Antigens, Neoplasm / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D017024:Chemotherapy, Adjuvant; D004273:DNA, Neoplasm / Q000032:analysis; D004067:Digestive System Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D005260:Female; D015321:Gene Rearrangement; D005803:Genes, Immunoglobulin; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D008198:Lymph Nodes / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D007143:Immunoglobulin Heavy Chains