Development of a modified prognostic index for patients with aggressive adult T-cell leukemia-lymphoma aged 70 years or younger: possible risk-adapted management strategies including allogeneic transplantation

Abstract

Adult T-cell leukemia-lymphoma is a distinct type of peripheral T-cell lymphoma caused by human T-cell lymphotropic virus type I. Although allogeneic stem cell transplantation after chemotherapy is a recommended treatment option for patients with aggressive adult T-cell leukemia-lymphoma, there is no consensus about indications for allogeneic stem cell transplantation because there is no established risk stratification system for transplant eligible patients. We conducted a nationwide survey of patients with aggressive adult T-cell leukemia-lymphoma in order to construct a new, large database that includes 1,792 patients aged 70 years or younger with aggressive adult T-cell leukemia-lymphoma who were diagnosed between 2000 and 2013 and received intensive first-line chemotherapy. We randomly divided patients into two groups (training and validation sets). Acute type, poor performance status, high soluble interleukin-2 receptor levels (> 5,000 U/mL), high adjusted calcium levels (≥ 12 mg/dL), and high C-reactive protein levels (≥ 2.5 mg/dL) were independent adverse prognostic factors used in the training set. We used these five variables to divide patients into three risk groups. In the validation set, median overall survival for the low-, intermediate-, and high-risk groups was 626 days, 322 days, and 197 days, respectively. In the intermediate- and high-risk groups, transplanted recipients had significantly better overall survival than non-transplanted patients. We developed a promising new risk stratification system to identify patients aged 70 years or younger with aggressive adult T-cell leukemia-lymphoma who may benefit from upfront allogeneic stem cell transplantation. Prospective studies are warranted to confirm the benefit of this treatment strategy.

Outcome of allogeneic hematopoietic cell transplantation in patients with adult T‐cell leukemia

“…Kawada et al performed a retrospective analysis of treatment outcome in ATL patients and showed improved 3‐year OS in allo‐HSCT recipients over those receiving chemotherapy alone. In a retrospective nationwide study, Fuji et al also recently reported better OS in transplanted patients compared with nontransplanted patients. The objective of our study was to retrospectively analyze the outcome of ATL patients undergoing allo‐HSCT at our institution.…”

“…In a retrospective nationwide study, Fuji et al 12 also recently reported better OS in transplanted patients compared with nontransplanted patients. The objective of our study was to retrospectively analyze the outcome of ATL patients undergoing allo-HSCT at our institution.…”

Role of up-front allogeneic hematopoietic stem cell transplantation for patients with aggressive adult T-cell leukemia-lymphoma: a decision analysis

“…Adult T-cell leukemia-lymphoma (ATL) is a distinct peripheral T-cell lymphoma caused by human T-cell lymphotropic virus type I with a dismal outcome [1][2][3][4]. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a promising treatment option for patients with aggressive ATL [3,5]. However, there is still no consensus whether all patients with aggressive ATL should undergo an up-front allo-HSCT.…”

“…However, there is still no consensus whether all patients with aggressive ATL should undergo an up-front allo-HSCT. Although retrospective analyses suggested the presence of survival benefit of up-front allo-HSCT compared with chemotherapy alone in patients with intermediate-or high-risk ATL [3], presence of significant selection bias made it difficult to assess the equipoise of the two treatments. A prospective randomized clinical trial (RCT) is desirable to clarify the benefit of up-front allo-HSCT, but it has never been conducted.…”

“…In order to address this problem, we here undertook a decision analysis using database the database of 1792 patients with aggressive ATL [3,6]. Decision analysis is a computerized modeling analysis that can simulate the clinical outcomes of different therapeutic strategies and identify an appropriate therapeutic strategy.…”