title: Mantle cell lymphoma, in leukaemic phase with prominent splenomegaly. A report of eight cases with similar clinical presentation and aggressive outcome.
abstract: Mantle cell lymphoma (MCL) is a well-defined peripheral B-cell lymphoma usually diagnosed upon peripheral lymph node biopsy. We report eight cases of peripheral B-cell leukaemia that demonstrate presumptive evidence of mantle cell characteristics. The patients had a median age of 68.5 years, and five were male. All presented with an enlarged spleen without any peripheral lymphadenopathies, and they were leukaemic at presentation (median lymphocytosis, 38x10(9)/l). Morphological diagnosis of MCL was very difficult in five cases but easier in three because we were able to analyse either pre- or post-mortem lymph nodes and spleen. The immunophenotype of blood lymphocytosis using flow cytometry, the presence of a t(11;14)(q13;q32) and a cyclin D1 expression by leukaemic cells all fit with the diagnosis of MCL. All patients progressed and died with a median overall survival of 8 months. Multifocal areas of transformation in blastoid or large cell variants were observed in the three autopsied patients. In summary, one should consider the diagnosis of MCL at presentation in leukaemic phase even in the absence of peripheral adenopathies.
authors.affiliation: Department of Pathology, H tel-Dieu de Paris, AP-HP, France. thierry.molina@htd-ap-hop-paris.fr
authors.forename: T J
authors.initials: TJ
authors.lastname: Molina
authors.forename: A
authors.initials: A
authors.lastname: Delmer
authors.forename: F
authors.initials: F
authors.lastname: Cymbalista
authors.forename: A
authors.initials: A
authors.lastname: Le Tourneau
authors.forename: J Y
authors.initials: JY
authors.lastname: Perrot
authors.forename: S
authors.initials: S
authors.lastname: Ramond
authors.forename: J P
authors.initials: JP
authors.lastname: Marie
authors.forename: J
authors.initials: J
authors.lastname: Audouin
authors.forename: R
authors.initials: R
authors.lastname: Zittoun
authors.forename: J
authors.initials: J
authors.lastname: Diebold
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D002878:Chromosomes, Human, Pair 1; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D019938:Cyclin D1 / Q000032:analysis; D020732:Cytogenetic Analysis; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D017353:Gene Deletion; D006801:Humans; D016130:Immunophenotyping; D015448:Leukemia, B-Cell / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology; D018655:Lymphocyte Count; D020522:Lymphoma, Mantle-Cell / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013163:Splenomegaly; D014178:Translocation, Genetic
chemical_list: D019938:Cyclin D1