title: Hepatosplenic gamma/delta T-cell lymphoma with isochromosome 7q, translocation t(7;21), and tetrasomy 8 in a 9-year-old girl.
abstract: The authors report a child younger than age 15 years with a rare hepatosplenic gamma/delta T-cell lymphoma, which is highly aggressive and primarily seen in young men. A 9-year-old girl presented with thrombocytopenia and hepatosplenomegaly. Bone marrow analysis revealed a metastatic pleomorphic lymphoma of peripheral T-cell phenotype, with rearrangement of the T-cell receptor gamma/delta and expression of CD3 and CD16/56. Instead of the previously reported primary, nonrandom, chromosomal abnormalities, isochromosome 7q and trisomy 8, this patient had four copies each of chromosome 7q, including isochromosome 7[i(7)(q10)] and der(21)t(7;21), as well as chromosome 8. This entity needs to be considered in women and children with lymphoma. Conventional therapy appears to be inadequate for cure.
authors: Divisions of Pediatric Hematology/Oncology, All Children's Hospital, University of South Florida, St. Petersburg, USA.
authors: Hans-Christoph
authors: HC
authors: Rossbach
authors: Wilfredo
authors: W
authors: Chamizo
authors: Doris P
authors: DP
authors: Dumont
authors: Jerry L
authors: JL
authors: Barbosa
authors: Maxine J
authors: MJ
authors: Sutcliffe
mesh_terms: D000782:Aneuploidy; D000951:Antigens, Neoplasm / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D017252:CD3 Complex / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D002648:Child; D002891:Chromosomes, Human, Pair 21 / Q000648:ultrastructure*; D002897:Chromosomes, Human, Pair 7 / Q000648:ultrastructure*; D002898:Chromosomes, Human, Pair 8; D005260:Female; D006529:Hepatomegaly / Q000209:etiology / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D018404:Isochromosomes; D008113:Liver Neoplasms / Q000188:drug therapy / Q000235:genetics* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000235:genetics* / Q000628:therapy; D009006:Monosomy; D014411:Neoplastic Stem Cells / Q000737:chemistry / Q000473:pathology; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis / Q000235:genetics*; D017452:Receptors, IgG / Q000032:analysis; D012074:Remission Induction; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000235:genetics* / Q000628:therapy; D013163:Splenomegaly / Q000209:etiology / Q000473:pathology; D014178:Translocation, Genetic; D014184:Transplantation, Homologous; D014314:Trisomy; D014960:X Chromosome
chemical_list: D000951:Antigens, Neoplasm; D017252:CD3 Complex; D019002:CD56 Antigen; D016692:Receptors, Antigen, T-Cell, gamma-delta; D017452:Receptors, IgG