title: Hepatosplenic T-cell lymphoma: clinicopathologic, immunophenotypic, and molecular characterization of 17 Chinese cases.
abstract: Hepatosplenic T-cell lymphoma is a highly aggressive tumor with a poor outcome. About 210 cases were identified through PubMed, of which fewer than 20 originated in Asia. We reviewed 17 Chinese cases of hepatosplenic T-cell lymphoma, including an unusual one with cutaneous pink papules, for clinicopathologic, immunophenotypic, and genotypic features; Epstein-Barr virus status; treatment; and outcome. The median age of the patients was 23 years. All patients had splenomegaly, and 88.2% of them had hepatomegaly as well. Bone marrow involvement was present in 53.3%. Eleven patients underwent splenectomy for diagnosis and treatment. Twelve specimens were collected by image-guided liver core biopsy or wedge resection. Histologically, the homogeneous small- to medium-size neoplastic lymphoid cells infiltrated the sinuses or sinusoids of the spleen, bone marrow, and liver. Diagnosis was based on image-guided core-needle biopsy of the liver alone in 41.2% of the cases. Immunohistochemically, 15 of the lesions were hepatosplenic γδ T-cell lymphoma, and the remaining 2 were hepatosplenic αβ T-cell lymphoma. Epstein-Barr virus was detected in both cases of hepatosplenic αβ T-cell lymphoma and one case of hepatosplenic γδ T-cell lymphoma. Most of the patients received cyclophosphamide, doxorubicin, vincristine, and prednisone therapy or regimens similar to it. Follow-up data were available in 70.6% of the patients; half of them died of the tumor, and the median survival time was 6 months. The frequency of bone marrow involvement was lower than that reported in the literature. Image-guided core-needle biopsy of the liver is recommended for diagnosis.
authors.affiliation: Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China.
authors.forename: Chang-li
authors.initials: CL
authors.lastname: Lu
authors.forename: Yuan
authors.initials: Y
authors.lastname: Tang
authors.forename: Qun-pei
authors.initials: QP
authors.lastname: Yang
authors.forename: Mi
authors.initials: M
authors.lastname: Wang
authors.forename: Sha
authors.initials: S
authors.lastname: Zhao
authors.forename: Cheng-feng
authors.initials: CF
authors.lastname: Bi
authors.forename: Neng-gang
authors.initials: NG
authors.lastname: Jiang
authors.forename: Wen-yan
authors.initials: WY
authors.lastname: Zhang
authors.forename: Jian-ping
authors.initials: JP
authors.lastname: Liu
authors.forename: Xia
authors.initials: X
authors.lastname: Xu
authors.forename: Wei-ping
authors.initials: WP
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D005838:Genotype; D006529:Hepatomegaly / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification; D006801:Humans; D016130:Immunophenotyping; D008099:Liver / Q000473:pathology; D008113:Liver Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy / Q000821:virology; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000473:pathology* / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D011948:Receptors, Antigen, T-Cell / Q000235:genetics / Q000378:metabolism; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D012189:Retrospective Studies; D013154:Spleen / Q000473:pathology; D013160:Splenic Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy / Q000821:virology; D013163:Splenomegaly / Q000473:pathology; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D011948:Receptors, Antigen, T-Cell; D016692:Receptors, Antigen, T-Cell, gamma-delta