title: Immunoarchitectural patterns in splenic marginal zone lymphoma: correlations with chromosomal aberrations, IGHV mutations, and survival. A study of 76 cases.
abstract: OBJECTIVE
  To describe 76 cases of splenic marginal zone lymphoma (SMZL), including correlations with clinical and other characteristics.
  
  
  RESULTS
  Patients were predominantly female, with a median age of 62 years. The main clinical presentation was splenomegaly, except for eight cases presenting with evolution of autoimmune disorders or spontaneous splenic rupture. White pulp infiltration was nodular, with a monophasic (42%) or biphasic (53%) pattern, and associated diffuse or nodular infiltration of the red pulp, except for four cases which had atrophic white pulp. Plasmacytic differentiation and the MYD88 L265P mutation were observed in 18% and 5% of the cases, respectively. Histological progression was considered in cases with a significant association of large cells with Ki67 > 30% and macronodular architecture (P = 0.001). Other significant correlations were found between del7q (44%) and del6q (17%) (P = 0.018), IGHV1-2*04 segment usage (35%) (P = 0.001) and unmutated IGHV (39%) (P = 0.019), and between CD5 expression (27%) and higher lymphocytosis (P = 0.002). Patients requiring intensive chemotherapy after splenectomy because of clinical and/or histological progression had significantly shorter overall survival (P = 0.012).
  
  
  CONCLUSIONS
  We report the histological spectrum of SMZL, and discuss the differential diagnosis and requirement for molecular and cytogenetic analysis in atypical cases.
authors.affiliation: Hospices Civils de Lyon, Service d'Anatomie Pathologique, Centre Hospitalier Lyon Sud, Université Lyon 1, Pierre Bénite, France. alexandra.traverse-glehen@chu-lyon.fr
authors.forename: Alexandra
authors.initials: A
authors.lastname: Traverse-Glehen
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Bachy
authors.forename: Lucile
authors.initials: L
authors.lastname: Baseggio
authors.forename: Evelyne
authors.initials: E
authors.lastname: Callet-Bauchu
authors.forename: Sophie
authors.initials: S
authors.lastname: Gazzo
authors.forename: Aurélie
authors.initials: A
authors.lastname: Verney
authors.forename: Sandrine
authors.initials: S
authors.lastname: Hayette
authors.forename: Laurent
authors.initials: L
authors.lastname: Jallades
authors.forename: Martine
authors.initials: M
authors.lastname: Ffrench
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.forename: Pascale
authors.initials: P
authors.lastname: Felman
authors.forename: Francoise
authors.initials: F
authors.lastname: Berger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D005260:Female; D050438:Genes, Immunoglobulin Heavy Chain; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics* / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009154:Mutation; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics; D011379:Prognosis; D013160:Splenic Neoplasms / Q000235:genetics* / Q000276:immunology* / Q000473:pathology
chemical_list: C507779:MYD88 protein, human; D053594:Myeloid Differentiation Factor 88