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


CONCLUSIONS
A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed.
Falchook
G S
GS
Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Vega
F
F
Dang
N H
NH
Samaniego
F
F
Rodriguez
M A
MA
Champlin
R E
RE
Hosing
C
C
Verstovsek
S
S
Pro
B
B
D000328:Adult; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D055815:Young Adult