1. Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases.
- Author
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Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y, Hu S, Bhagat G, Oki Y, Patel KP, Routbort M, Luthra R, Fanale MA, Bueso-Ramos CE, Jorgensen JL, Vega F, Chen W, Hoehn D, Konoplev S, Milton DR, Wistuba I, Li S, You MJ, Young KH, and Miranda RN
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Biomarkers, Tumor analysis, Biomarkers, Tumor genetics, Bone Marrow pathology, Bone Marrow Examination, Child, Child, Preschool, Chromosomes, Human, Pair 7, Chromosomes, Human, Pair 8 genetics, Disease-Free Survival, Female, Genetic Predisposition to Disease, Hepatomegaly pathology, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Isochromosomes, Kaplan-Meier Estimate, Male, Middle Aged, Phenotype, Proportional Hazards Models, Receptors, Antigen, T-Cell, alpha-beta genetics, Receptors, Antigen, T-Cell, alpha-beta immunology, Receptors, Antigen, T-Cell, gamma-delta genetics, Receptors, Antigen, T-Cell, gamma-delta immunology, Retrospective Studies, Risk Factors, Splenomegaly pathology, Therapeutics, Time Factors, Trisomy genetics, United States, Young Adult, Liver Neoplasms drug therapy, Liver Neoplasms genetics, Liver Neoplasms immunology, Liver Neoplasms mortality, Liver Neoplasms pathology, Lymphoma, T-Cell drug therapy, Lymphoma, T-Cell genetics, Lymphoma, T-Cell immunology, Lymphoma, T-Cell mortality, Lymphoma, T-Cell pathology, Splenic Neoplasms drug therapy, Splenic Neoplasms genetics, Splenic Neoplasms immunology, Splenic Neoplasms mortality, Splenic Neoplasms pathology
- 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.
- Published
- 2016
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