Hematopoietic stem cell transplantation for T-cell large granular lymphocyte leukemia: a retrospective study of the European society for blood and marrow transplantation

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Date: May 2016
From: Leukemia(Vol. 30, Issue 5)
Publisher: Nature Publishing Group
Document Type: Letter to the editor; Report
Length: 2,162 words
Lexile Measure: 1650L

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Large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disorder characterized by clonal expansion of either [CD3.sup.+] cytotoxic T-lymphocytes or [CD3.sup.-] NK cells. (1) WHO (World Health Organization) classification has recognized LGL leukemia as a specific entity among the subgroup of mature peripheral T-cell neoplasms. Clinical presentation is dominated by splenomegaly, neutropenia with recurrent infections and autoimmune manifestations. (2) Due to low incidence and thus the lack of prospective studies, no standard treatment has been established in LGL leukemia. Although usually considered as an indolent disease, most patients will require therapy based on immunosuppressive drugs such as methotrexate, cyclophosphamide or cyclosporin. (3) Despite of a good clinical response rate, long-term remissions are rarely observed. Patients who are refractory to immunosuppressive therapy or with initial aggressive clinical course should be offered alternative strategies because combined chemotherapy regimens such as cyclophosphamide-doxorubicine-vincristine-prednisone (CHOP)-like regimens gives disappointing results. (3) Few cases of aggressive LGL-leukemia have been described with an overall survival (OS) not exceeding 2 years. (5) In this serious situation, intensification of therapy by high-dose chemotherapy may be considered. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only curative treatment for several immune and hematologic disorders but is associated with a high toxicity mainly due to graft-versus-host disease (GVHD). Graft-versus-lymphoma (GVL) effects have been proven in various lymphoproliferative disorders. (6) However, the feasibility and efficacy of allo-HSCT in LGL leukemia has not been evaluated so far and data are only limited to few sporadic case reports. (7,8) Autologous HSCT (auto-HSCT) is associated with prolonged progression-free survival (PFS) in several lymphohematopoietic malignancies and represents a valuable option in peripheral T-cell lymphoma. (9) However, the value of auto-HSCT in relapsed or refractory LGL leukemia has not been evaluated to date. In this letter, we report the outcome of 15 patients treated by auto-HSCT or allo-HSCT for T-cell LGL leukemia and reported to the European Society for Bone and Marrow Transplantation (EBMT) registry.

This is a registry-based retrospective multicenter study including patients 18 years or above who underwent allo-HSCT or auto-HSCT for T-cell LGL leukemia between 1 January 2004 and 30 November 2011 while cases were reported to the EBMT. EBMT is a voluntary organization comprising >600 transplant centers mainly from Europe. Accreditation as a member center requires submission of minimal essential data-A form (www.ebmt.org) from all consecutive patients to a central registry. Informed consent for transplantation and data collection were obtained locally according to regulations applicable at the time of transplantation. Baseline patient, disease and transplant data were collected from minimal essential data-A forms. In addition, a questionnaire was sent out to collect additional data including autoimmune disease, clinical presentation, before transplantation administered therapies and updated follow-up information. Centers were then contacted to provide written flow-cytometry diagnostic reports to confirm the diagnosis of LGL leukemia. Conditioning regimen intensity was defined according to definition recently proposed. (10,11) GVHD was graded according to consensus criteria. (12,13) The primary end points studied were 2-year OS and 2-year PFS. Secondary end points were incidence of disease relapse or progression...

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Gale Document Number: GALE|A454428346