Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases. (May 2016)

• Record Type: Journal Article
• Authors: Yabe, Mariko; Medeiros, L. Jeffrey; Tang, Guilin; Wang, Sa A.; Ahmed, Sairah; Nieto, Yago; Hu, Shimin; Bhagat, Govind; Oki, Yasuhiro; Patel, Keyur P.; Routbort, Mark; Luthra, Rajyalakshmi; Fanale, Michelle A.; Bueso-Ramos, Carlos E.; Jorgensen, Jeffrey L.; Vega, Francisco; Chen, Weina; Hoehn, Daniela; Konoplev, Sergej; Milton, Denai R.; Wistuba, Ignacio; Li, Shaoying; You, M. James; Young, Ken H.; Miranda, Roberto N.
• Is Part Of: American journal of surgical pathology. Volume 40:Number 5(2016)
• Publication Date: 2016-05
• Subjects: hepatosplenic T-cell lymphoma -- prognostic factors -- stem cell transplant; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000000614
• Languages: English
• ISSNs: 0147-5185

Abstract

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.