Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience

Hepatosplenic T-cell lymphoma (HSTCL) is an aggressive subtype of peripheral T-cell lymphoma. Prospective trials providing therapeutic guidance are lacking as a result of this disease's rarity. We studied the clinical characteristics and therapeutic outcomes of 22 patients with HSTCL. Liver involvement and chronic immunosuppression before diagnosis were associated with worse prognosis. Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. Patients and Methods: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. Results: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). Conclusion: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size. (C) 2020 Elsevier Inc. All rights reserved.

Automated summary

Hepatosplenic T-cell lymphoma is a rare and aggressive subtype of peripheral T-cell lymphoma with poor prognosis. Liver involvement and chronic immunosuppression are associated with worse survival outcomes in patients with HSTCL. Although splenectomy and allogeneic hematopoietic cell transplant have anecdotal benefit, larger studies are needed to determine their effectiveness.