Relapsed or Refractory Lymphoblastic Lymphoma in Children: Results and Analysis of 23 Patients in the EORTC 58951 and the LMT96 Protocols

Abstract

for the SFCE and the EORTC children leukemia group Background. The treatment of children with T-cell lymphoblastic lymphoma (T-LBL) and precursor B-cell lymphoblastic lymphoma (pB-LBL) has improved during the last decades. However, patients with relapsed or refractory lymphomas still have a poor prognosis. Methods. We report the characteristics and evolution of T-LBL and pB-LBL relapses in two multicenter prospective studies (LMT 96, European Organization for Research and Treatment of Cancer 58951). Results. From 1997From to 2008 patients were included in these studies (157 T-LBL; 37 pB-LBL); among them, 23 patients underwent relapse or progression (18 T-LBL and 5 pB-LBL). The median age was 7.7 years (range 1.4-16.3). The survival rate at 8 years was 8.7% (21 deaths). The median time from diagnosis to relapse was 9 months [1-69] and 11 months for T-LBL and pB-LBL, respectively. Twenty-two patients received a second-line treatment but remission was achieved in only seven patients. In 10 patients, intensification with hematopoietic stem cell transplantation (HSCT) was performed and four of them had a second relapse. Two patients still alive had T-LBL, experienced relapses 15 and 69 months after diagnosis, and received HSCT. Relapse during the intensive phase and second-line treatment without HSCT were identified as risk factors for bad prognosis (P = 0.01). Conclusions. The results of second-line treatment, including intensive chemotherapy and HSCT, show that salvage treatment is still disappointing in controlling refractory forms. Early identification of patients at high risk of relapse is mandatory, allowing earlier intensification. Valid prognostic parameters, such as biological markers, are needed. International cooperation is warranted to collect more data on these rare diagnoses. Pediatr

Lymphoblastic lymphoma in children and adolescents: review of current challenges and future opportunities

“…Great strides have been made in the treatment of lymphoblastic lymphoma (LBL) with event‐free and overall‐survivals exceeding 80% (Termuhlen et al , ; Landmann et al , ). However, cure rates for patients with relapsed or refractory disease remain dismal (Schmidt & Burkhardt, ; Michaux et al , ). The vast majority is of T‐lymphoblastic origin (T‐LBL, 70–80%) with only 20–25% arising from B lymphoblasts (pB‐LBL) and mixed myeloid/lymphoblastic (MPAL) phenotypes being very rare (Burkhardt et al , ; Oschlies et al , ).…”

Novel targeted therapeutic agents for the treatment of childhood, adolescent and young adult non‐Hodgkin lymphoma

“…Other candidates for prospective evaluation may include the kinetics of the response to treatment. The outcome with r/r disease is dismal with a cure rate generally below 30% (Michaux et al , ). In LL, there is a clear indication for an allograft if a second complete remission (CR2) is achieved.…”

The effect of graft‐versus‐host disease on outcomes after allogeneic stem cell transplantation for refractory lymphoblastic lymphoma in children and young adults

“…The treatment of children with lymphoblastic lymphoma (LBL) has improved, such that event‐free survival (EFS) has reached 80% to 90% during the past two decades with intensive combinatorial chemotherapy . However, patients with relapsed or refractory LBL continue to have a poor prognosis . In particular, allogeneic transplantation is necessary for survival in most patients with relapsed or refractory LBL.…”

“…1 However, patients with relapsed or refractory LBL continue to have a poor prognosis. [2][3][4] In particular, allogeneic transplantation is necessary for survival in most patients with relapsed or refractory LBL. Allogeneic blood stem cell transplantation (SCT) has some beneficial effect for these patients, but detailed information regarding its efficiency remains limited.…”