Haematopoietic stem cell transplantation in hepatosplenic T-cell lymphoma: a retrospective analysis of the EBMT Lymphoma Working Party

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BACKGROUND: Hepatosplenic T-cell lymphoma is a rare and aggressive lymphoma with no established standard therapy. Chemotherapy is generally associated with poor outcomes. We aimed to better define the roles of allogeneic haematopoietic stem cell transplantation (allo-HSCT) and autologous HSCT (auto-HSCT) in patients with hepatosplenic T-cell lymphoma. METHODS: We conducted an analysis of patients aged 18 years or older who were registered with the European Society for Blood and Bone Marrow Transplantation (EBMT) and cooperating Asian centres. 64 centres from Europe and Asia contributed patients who received allo-HSCT and auto-HSCT. For this analysis, a database query of the registry was performed, and the necessary information, such as baseline characteristics, previous treatments, transplantation data, and follow-up data, was collected via a survey sent to all participating centres. Hepatosplenic T-cell lymphoma was diagnosed by local pathologists, and patients with a diagnosis (confirmed or consistent with the disease) were included. The main outcomes were progression-free survival, overall survival, non-relapse mortality, and relapse incidence at 3 years from HSCT. FINDINGS: 94 patients (median age 36 years [IQR 28-46]; 65 [69%] males and 29 [31%] females) who underwent allo-HSCT between Dec 1, 2005, and Jan 29, 2024, and 27 patients (37 years [29-53]; 17 [63%] males and ten [37%] females) who underwent auto-HSCT between Aug 20, 2004, and Feb 8, 2022, were included. 95 (83%) of 115 patients were from Europe and 20 (17%) were from Asia. 57 (47%) of 121 patients received first-line therapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone or variants. The majority of patients were in complete remission when they underwent transplantation: 41 (44%) of 93 patients with remission data before allo-HSCT and 20 (74%) of 27 patients before auto-HSCT. With a median follow-up of 4·5 years (IQR 3·4-5·6) in patients who underwent allo-HSCT, 3-year progression-free survival was 50·5% (95% CI 39·4-60·5) and 3-year overall survival was 55·0% (43·9-64·8). With a median follow-up of 5·0 years (IQR 4·3-6·6) for patients treated with auto-HSCT, 3-year progression-free survival was 38·9% (95% CI 17·5-60·0) and 3-year overall survival was 63·5% (41·3-79·3). 3-year non-relapse mortality was 11·7% (95% CI 5·9-19·5) and the 3-year relapse incidence was 37·9% (27·6-48·1) for patients who underwent allo-HSCT. The 3-year non-relapse mortality was 11·1% (95% CI 1·7-30·7) and the 3-year relapse incidence was 50·0% (24·9-70·8) for patients who underwent auto-HSCT. INTERPRETATION: We showed that allo-HSCT is an effective and potentially curative treatment option for patients with hepatosplenic T-cell lymphoma; auto-HSCT is frequently followed by relapse and might be only recommended to patients with a complete response and ineligible for allo-HSCT. FUNDING: None.

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