title: A γ/δ T-cell receptor prolymphocytic leukemia and CD4-/CD8- double-negative immunophenotype in a pediatric patient.
abstract: T-cell prolymphocytic leukemia is a very rare neoplasm, peaking in the seventh decade. An extensive search failed to find any report of this malignancy in the pediatric population. The malignant cell is morphologically characterized by a high nucleocytopasmic ratio, condensed chromatin, a single nucleolus, and nongranular basophilic cytoplasm. Cells are usually positive for the α/β and only rarely to the γ/δ T-cell receptors. Most patients follow an aggressive clinical course, only some respond to anti-CD52. We present a 6-year-old boy with T-cell prolymphocytic leukemia. The malignant cells expressed a postthymic immunophenotype (CD4/CD8) and positivity for the γ/δ T-cell receptors. The child died after 8 months despite aggressive chemotherapy, anti-CD52, and an allogeneic bone marrow transplant.
authors.affiliation: *Department of Pediatric Hematology/Oncology, Soroka Medical Center †Department of Pediatric Hematology/Oncology, Medical School for International Health, Soroka Medical Center ‡Flow Cytometry Unit, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Be'er Sheva, Israel.
authors.forename: Asher M
authors.initials: AM
authors.lastname: Moser
authors.forename: Abed A
authors.initials: AA
authors.lastname: Quider
authors.forename: Jeremiah A
authors.initials: JA
authors.lastname: Groen
authors.forename: George
authors.initials: G
authors.lastname: Shubinsky
authors.forename: Joseph
authors.initials: J
authors.lastname: Kapelushnik
mesh_terms: D015703:Antigens, CD / Q000032:analysis*; D016827:CD8 Antigens / Q000032:analysis*; D002648:Child; D006801:Humans; D016130:Immunophenotyping; D015461:Leukemia, Prolymphocytic, T-Cell / Q000276:immunology*; D008297:Male; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*
chemical_list: D015703:Antigens, CD; D016827:CD8 Antigens; D016692:Receptors, Antigen, T-Cell, gamma-delta