title: Composite mantle cell lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: a clinicopathologic and molecular study.
abstract: Mantle cell lymphoma (MCL) and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) share many features and both arise from CD5+ B-cells; their distinction is critical as MCL is a more aggressive neoplasm. Rarely, cases of composite MCL and CLL/SLL have been reported. Little is known about the nature of these cases and, in particular, the clonal relationship of the 2 lymphomas. Eleven composite MCL and CLL/SLL cases were identified. The clinical, morphologic and immunophenotypic features of the MCL and CLL/SLL were characterized. IGH (immunoglobulin heavy chain) gene analysis was performed on microdissected MCL and CLL/SLL components to assess their clonal relationship. Ten patients had lymphadenopathy, and 7 patients had bone marrow involvement. The MCL component had the following growth patterns: in situ (n = 1), mantle zone (n = 3), nodular and diffuse (n = 3), diffuse (n = 3), and interstitial in the bone marrow (the only patient without lymphadenopathy) (n = 1); 6 MCLs had blastoid or pleomorphic and 5 small lymphocytic features. The CLL/SLL component was nodular (n = 9) or diffuse (n = 2). All MCL were CD5(+) and cyclin D1(+) with t(11;14) translocation. All CLL/SLL were CD5(+), CD23(+) and negative for cyclin D1 or t(11;14). IGH gene analysis showed that the MCL and CLL/SLL components displayed different sized fragments, indicating that the MCL and CLL/SLL are likely derived from different neoplastic B-cell clones. The lack of a clonal relationship between the MCL and CLL/SLL components suggests that MCL and CLL/SLL components represent distinct disease processes and do not share a common progenitor B-cell.
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77230-1439, USA.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Hoeller
authors.forename: Yi
authors.initials: Y
authors.lastname: Zhou
authors.forename: Rashmi
authors.initials: R
authors.lastname: Kanagal-Shamanna
authors.forename: Zijun Y
authors.initials: ZY
authors.lastname: Xu-Monette
authors.forename: Daniela
authors.initials: D
authors.lastname: Hoehn
authors.forename: Michel
authors.initials: M
authors.lastname: Bihl
authors.forename: Steven H
authors.initials: SH
authors.lastname: Swerdlow
authors.forename: Andreas
authors.initials: A
authors.lastname: Rosenwald
authors.forename: German
authors.initials: G
authors.lastname: Ott
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.forename: Cherie H
authors.initials: CH
authors.lastname: Dunphy
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bueso-Ramos
authors.forename: Pei
authors.initials: P
authors.lastname: Lin
authors.forename: Michael
authors.initials: M
authors.lastname: Wang
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
authors.forename: Alexander
authors.initials: A
authors.lastname: Tzankov
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Ken H
authors.initials: KH
authors.lastname: Young
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D058617:Composite Lymphoma / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D005260:Female; D015322:Gene Rearrangement, B-Lymphocyte / Q000235:genetics; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D020522:Lymphoma, Mantle-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged
chemical_list: D007143:Immunoglobulin Heavy Chains