title: Human T-cell lymphotropic virus-1-positive T-cell leukemia/lymphoma in a child. Report of a case and review of the literature.
abstract: Adult T-cell leukemia/lymphoma is a monoclonal T-cell neoplasm associated with human T-cell lymphotropic virus-1 (HTLV-1) that occurs almost exclusively in adults. This report concerns a Romanian girl who had recurrent skin eruptions since infancy, subcutaneous tumors in childhood, and peripheral blood lymphocytosis, which initially developed at the age of 12 years. The circulating lymphocytes were of helper T-cell immunophenotype. Serologic studies demonstrated a number of HTLV-1 antigens in the child and her mother, and molecular analyses revealed monoclonal T-cell-receptor gamma gene rearrangement and detectable HTLV-1 proviral DNA. Conventional cytogenetic studies revealed a t(3;6)(q23;q27) chromosome translocation in most of the neoplastic cells. The patient initially responded well to interferon alfa therapy and showed regression of skin lesions and diminished lymphocytosis, but 4 years later, she developed massive lymphadenopathy and leukemic infiltration of the breast. At last clinical follow-up, at the age of 17 years, the patient had stable low-level peripheral lymphocytosis and subcutaneous tumors while being continuously treated with interferon alfa. Our review of the literature revealed six additional children with HTLV-1-associated T-cell leukemia/lymphoma, including one case with a similar clinical presentation and ethnic background. To our knowledge, the t(3;6)(q23;q27) translocation identified in this patient's neoplasm has not been previously reported in adult T-cell leukemia/lymphoma cases and may explain the early onset of disease. Although adult T-cell leukemia/lymphoma is rare in Romania, the identification of healthy carriers and vertical transmission raise the possibility that Romania might be an endemic region for HTLV-1 infection.
authors.affiliation: Division of Anatomic Pathology, City of Hope National Medical Center, Duarte, Calif. 91010, USA.
authors.forename: B T
authors.initials: BT
authors.lastname: Lin
authors.forename: M
authors.initials: M
authors.lastname: Musset
authors.forename: A M
authors.initials: AM
authors.lastname: Székely
authors.forename: J
authors.initials: J
authors.lastname: Alexandre
authors.forename: S
authors.initials: S
authors.lastname: Fraitag
authors.forename: C
authors.initials: C
authors.lastname: Bodemer
authors.forename: A
authors.initials: A
authors.lastname: Charpentier
authors.forename: N
authors.initials: N
authors.lastname: Frenoy
authors.forename: J L
authors.initials: JL
authors.lastname: Misset
authors.forename: L J
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: H
authors.initials: H
authors.lastname: Rappaport
mesh_terms: D001483:Base Sequence; D002353:Carrier State / Q000175:diagnosis / Q000453:epidemiology; D002648:Child; D004279:DNA, Viral / Q000032:analysis / Q000737:chemistry / Q000235:genetics; D015485:Deltaretrovirus Antigens / Q000032:analysis; D005260:Female; D015490:HTLV-I Infections / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D015368:Human T-lymphotropic virus 1 / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D016130:Immunophenotyping; D016898:Interferon-alpha / Q000627:therapeutic use; D007621:Karyotyping; D015458:Leukemia, T-Cell / Q000175:diagnosis* / Q000473:pathology / Q000821:virology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000473:pathology / Q000821:virology*; D012383:Romania / Q000453:epidemiology; D012867:Skin / Q000737:chemistry / Q000473:pathology; D014178:Translocation, Genetic
chemical_list: D004279:DNA, Viral; D015485:Deltaretrovirus Antigens; D016898:Interferon-alpha