TY - JOUR
T1 - Prognostic factors of hepatosplenic T-cell lymphoma clinicopathologic study of 28 cases
AU - Yabe, Mariko
AU - Medeiros, L. Jeffrey
AU - Tang, Guilin
AU - Wang, Sa A.
AU - Ahmed, Sairah
AU - Nieto, Yago
AU - Hu, Shimin
AU - Bhagat, Govind
AU - Oki, Yasuhiro
AU - Patel, Keyur P.
AU - Routbort, Mark
AU - Luthra, Rajyalakshmi
AU - Fanale, Michelle A.
AU - Bueso-Ramos, Carlos E.
AU - Jorgensen, Jeffrey L.
AU - Vega, Francisco
AU - Chen, Weina
AU - Hoehn, Daniela
AU - Konoplev, Sergej
AU - Milton, Denai R.
AU - Wistuba, Ignacio
AU - Li, Shaoying
AU - You, M. James
AU - Young, Ken H.
AU - Miranda, Roberto N.
N1 - Publisher Copyright:
© Copyright 2016 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2016
Y1 - 2016
N2 - 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, ab 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.
AB - 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, ab 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.
KW - Hepatosplenic T-cell lymphoma
KW - Prognostic factors
KW - Stem cell transplant
UR - http://www.scopus.com/inward/record.url?scp=84957921599&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84957921599&partnerID=8YFLogxK
U2 - 10.1097/PAS.0000000000000614
DO - 10.1097/PAS.0000000000000614
M3 - Article
C2 - 26872013
AN - SCOPUS:84957921599
SN - 0147-5185
VL - 40
SP - 676
EP - 688
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 5
ER -