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Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases.

Publication ,  Journal Article
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; Li, S ...
Published in: Am J Surg Pathol
May 2016

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.

Duke Scholars

Published In

Am J Surg Pathol

DOI

EISSN

1532-0979

Publication Date

May 2016

Volume

40

Issue

5

Start / End Page

676 / 688

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Trisomy
  • Time Factors
  • Therapeutics
  • Splenomegaly
  • Splenic Neoplasms
  • Risk Factors
  • Retrospective Studies
  • Receptors, Antigen, T-Cell, gamma-delta
 

Citation

APA
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ICMJE
MLA
NLM
Yabe, M., Medeiros, L. J., Tang, G., Wang, S. A., Ahmed, S., Nieto, Y., … Miranda, R. N. (2016). Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases. Am J Surg Pathol, 40(5), 676–688. https://doi.org/10.1097/PAS.0000000000000614

Published In

Am J Surg Pathol

DOI

EISSN

1532-0979

Publication Date

May 2016

Volume

40

Issue

5

Start / End Page

676 / 688

Location

United States

Related Subject Headings

  • Young Adult
  • United States
  • Trisomy
  • Time Factors
  • Therapeutics
  • Splenomegaly
  • Splenic Neoplasms
  • Risk Factors
  • Retrospective Studies
  • Receptors, Antigen, T-Cell, gamma-delta