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Annals of Oncology Advance Access originally published online on February 23, 2009
Annals of Oncology 2009 20(6):1080-1085; doi:10.1093/annonc/mdn751
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© The Author 2009. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oxfordjournals.org

hematologic malignancies

Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment

G. S. Falchook1, F. Vega2, N. H. Dang1, F. Samaniego1, M. A. Rodriguez1, R. E. Champlin3, C. Hosing3, S. Verstovsek4 and B. Pro1,*

1 Department of Lymphoma/Myeloma
2 Department of Hematopathology
3 Department of Stem Cell Transplantation and Cellular Therapy
4 Department of Leukemia, The University of Texas M.D. Anderson Cancer Center, Houston, USA

* Correspondence to: Dr B. Pro, Department of Lymphoma/Myeloma, Unit 429, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA. Tel: +1+713-792-2860; Fax: +1-713-794-5656; E-mail: bpro{at}mdanderson.org

Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare peripheral T-cell lymphoma; treatment with standard anthracycline-containing chemotherapy regimens has been disappointing, and an optimal treatment strategy for this patient population has not yet been determined.

Methods: We identified 15 cases of pathologically confirmed HSTCL in the institution's database. Clinical characteristics and treatment results were reviewed.

Results: Complete responses (CRs) were achieved in 7 of 14 patients who received chemotherapy. Achievement of CR was followed by hematopoietic stem-cell transplantation in three patients. Median duration of CR was 8 months (range 2 to 32+ months) with four patients currently alive and in CR at 5, 8, 12, and 32 months, respectively. Median overall survival (OS) was 11 months (range 2 to 36+ months). Patients who achieved a CR had a median OS of 13 months, compared with 7.5 months in patients who did not achieve a CR. Risk factors associated with worse outcome included male gender, failure to achieve a CR, history of immunocompromise, and absence of a T-cell receptor gene rearrangement in the gamma chain.

Conclusion: A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed.

Key words: clinicopathological features, hematopoietic stem-cell transplantation, hepatosplenic T-cell lymphoma, treatment

Received for publication November 11, 2008. Accepted for publication November 19, 2008.


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