title: Childhood de novo CD5+ Diffuse Large B-cell Lymphoma: a Separate Entity?
abstract: De novo CD5-positive diffuse large B-cell lymphoma (CD5+ DLBCL) is a subtype of DLBCL found predominantly in older individuals. This particular subtype has been associated with a female predominance and a more aggressive clinical course. Conversely, this entity has not been described in the pediatric population. We report a case of a 12 year-old boy who presented with an ileocecal intussusception. Radiologic, morphologic, and immunophenotypic analysis revealed an isolated extranodal mass consistent with a CD5+ DLBCL, germinal center cell phenotype. Fluorescent in situ hybridization analysis was negative for cMYC, BCL6, BCL2, MLL, and IGH/CCND1 rearrangement and showed loss of one copy of MLL in 32% cells. The patient was treated with four cycles of cyclophosphamide, vincristine, prednisolone, methotrexate, and doxorubicin and achieved complete remission. To the best of our knowledge, this is the first detailed report of a de novo CD5+ DLBCL occurring in a child.
authors.affiliation: Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA amc2343@columbia.edu.
authors.forename: Amy
authors.initials: A
authors.lastname: Coffey
authors.affiliation: Department of Pathology and Cell Biology, Divison of Hematopathology, Columbia University Medical Center, New York, NY, USA Department of Anatomical and Clinical Pathology, Cooper University Medical Center, New York, NY, USA.
authors.forename: Ashleigh
authors.initials: A
authors.lastname: Allen
authors.affiliation: Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA Department of Pathology and Cell Biology, Divison of Hematopathology, Columbia University Medical Center, New York, NY, USA.
authors.forename: Matthew B
authors.initials: MB
authors.lastname: Thomsen
authors.affiliation: Department of Pediatrics, Division of Hematology and Oncology, Columbia University Medical Center, New York, NY, USA.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Sulis
authors.affiliation: Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA.
authors.forename: Vundavalli
authors.initials: V
authors.lastname: Murty
authors.affiliation: Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA Department of Pathology and Cell Biology, Divison of Hematopathology, Columbia University Medical Center, New York, NY, USA.
authors.forename: Daniela
authors.initials: D
authors.lastname: Hoehn
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018956:CD5 Antigens / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D019938:Cyclin D1 / Q000235:genetics; D015321:Gene Rearrangement; D011495:Histone-Lysine N-Methyltransferase / Q000235:genetics; D006801:Humans; D007082:Ileum / Q000473:pathology; D017404:In Situ Hybridization, Fluorescence; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D008297:Male; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics; D055815:Young Adult
chemical_list: C530104:CCND1 protein, human; D018956:CD5 Antigens; C497422:KMT2A protein, human; D019938:Cyclin D1; D051788:Myeloid-Lymphoid Leukemia Protein; D011495:Histone-Lysine N-Methyltransferase