title: Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases.
abstract: Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of lymphoma. Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by lymphoma. The HSTCL cells expressed γδ T-cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). Conventional cytogenetics and/or fluorescence in situ hybridization analysis in 24 patients at diagnosis showed isochromosome 7q (i7q) in 10 (42%) and trisomy 8 in 8 (33%) patients. Median overall survival (OS) and event-free survival (EFS) were each 28.3 months. Serum bilirubin level ≥1.5 mg/dL, αβ TCR expression, and trisomy 8 each correlated significantly with shorter OS and EFS. Patients with HSTCL received a variety of chemotherapy regimens with no regimen better than any other. However, patients who underwent stem cell transplant showed longer survival (OS: hazard ratio 0.3, P=0.09; EFS: hazard ratio 0.2, P=0.034). In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αβ TCR expression, and trisomy 8 identifying a poorer prognostic group. In addition, the outcomes of this patient cohort suggest that stem cell transplantation has value for the treatment of patients with HSTCL.
authors.affiliation: Departments of *Hematopathology †Stem Cell Transplantation §Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX #Biostatistics **Translational and Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston ¶Department of Pathology, UT Southwestern Medical Center, Dallas, TX ‡Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY ∥Department of Pathology, University of Miami, Miami, FL.
authors.forename: Mariko
authors.initials: M
authors.lastname: Yabe
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Guilin
authors.initials: G
authors.lastname: Tang
authors.forename: Sa A
authors.initials: SA
authors.lastname: Wang
authors.forename: Sairah
authors.initials: S
authors.lastname: Ahmed
authors.forename: Yago
authors.initials: Y
authors.lastname: Nieto
authors.forename: Shimin
authors.initials: S
authors.lastname: Hu
authors.forename: Govind
authors.initials: G
authors.lastname: Bhagat
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Oki
authors.forename: Keyur P
authors.initials: KP
authors.lastname: Patel
authors.forename: Mark
authors.initials: M
authors.lastname: Routbort
authors.forename: Rajyalakshmi
authors.initials: R
authors.lastname: Luthra
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Fanale
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bueso-Ramos
authors.forename: Jeffrey L
authors.initials: JL
authors.lastname: Jorgensen
authors.forename: Francisco
authors.initials: F
authors.lastname: Vega
authors.forename: Weina
authors.initials: W
authors.lastname: Chen
authors.forename: Daniela
authors.initials: D
authors.lastname: Hoehn
authors.forename: Sergej
authors.initials: S
authors.lastname: Konoplev
authors.forename: Denai R
authors.initials: DR
authors.lastname: Milton
authors.forename: Ignacio
authors.initials: I
authors.lastname: Wistuba
authors.forename: Shaoying
authors.initials: S
authors.lastname: Li
authors.forename: M James
authors.initials: MJ
authors.lastname: You
authors.forename: Ken H
authors.initials: KH
authors.lastname: Young
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001853:Bone Marrow / Q000473:pathology; D001856:Bone Marrow Examination; D002648:Child; D002675:Child, Preschool; D002897:Chromosomes, Human, Pair 7; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D018572:Disease-Free Survival; D005260:Female; D020022:Genetic Predisposition to Disease; D006529:Hepatomegaly / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D018404:Isochromosomes; D053208:Kaplan-Meier Estimate; D008113:Liver Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D016016:Proportional Hazards Models; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics / Q000276:immunology; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics / Q000276:immunology; D012189:Retrospective Studies; D012307:Risk Factors; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology; D013163:Splenomegaly / Q000473:pathology; D013812:Therapeutics; D013997:Time Factors; D014314:Trisomy / Q000235:genetics; D014481:United States; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta