title: Double- and triple-hit lymphomas can present with features suggestive of immaturity, including TdT expression, and create diagnostic challenges.
abstract: Double- and triple-hit lymphomas (DHL/THL) are aggressive B-cell neoplasms characterized by translocation of MYC with concurrent BCL2 and/or BCL6 translocation. In this retrospective study from one institution, we report clinicopathologic features of 13 cases (9 DHL/4 THL). The median age was 59 years (range 30-74) and patients included eight females and five males. Presentation included enlarging lymphadenopathy/masses (11 patients) and abnormal peripheral blood findings (2 patients). Features which raised the differential of an immature neoplasm included terminal deoxynucleotidyl transferase positivity (four cases, two THL/two DHL); dim CD45 expression (seven cases), lack of CD20 (two cases), or lack of surface immunoglobulin light chain (three cases) by flow cytometry; and blastoid morphology (two cases). We conclude that expression of TdT in a B-cell lymphoma with mature features or expression of surface light chain in a case otherwise suggestive of B-lymphoblastic leukemia/lymphoma should prompt an expedited evaluation for DHL/THL.
authors.affiliation: a Department of Laboratory Medicine and Pathology , University of Minnesota , Minneapolis , MN , USA ;
authors.forename: Laura
authors.initials: L
authors.lastname: Moench
authors.affiliation: b Department of Medicine, Division of Hematology, Oncology, and Transplantation , University of Minnesota , Minneapolis , MN , USA.
authors.forename: Zohar
authors.initials: Z
authors.lastname: Sachs
authors.affiliation: a Department of Laboratory Medicine and Pathology , University of Minnesota , Minneapolis , MN , USA ;
authors.forename: Garth
authors.initials: G
authors.lastname: Aasen
authors.affiliation: a Department of Laboratory Medicine and Pathology , University of Minnesota , Minneapolis , MN , USA ;
authors.forename: Michelle
authors.initials: M
authors.lastname: Dolan
authors.affiliation: a Department of Laboratory Medicine and Pathology , University of Minnesota , Minneapolis , MN , USA ;
authors.forename: Vanessa
authors.initials: V
authors.lastname: Dayton
authors.affiliation: a Department of Laboratory Medicine and Pathology , University of Minnesota , Minneapolis , MN , USA ;
authors.forename: Elizabeth L
authors.initials: EL
authors.lastname: Courville
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor; D002471:Cell Transformation, Neoplastic / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D004253:DNA Nucleotidylexotransferase / Q000235:genetics* / Q000378:metabolism; D005260:Female; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009367:Neoplasm Staging; D012189:Retrospective Studies
chemical_list: D014408:Biomarkers, Tumor; D004253:DNA Nucleotidylexotransferase
keywords: B lymphoblastic leukemia/lymphoma; B-cell lymphoma unclassifiable; diffuse large B-cell lymphoma; double-hit lymphoma; triple-hit lymphoma