Relapsed or Refractory Lymphoblastic Lymphoma in Children: Results and Analysis of 23 Patients in the EORTC 58951 and the LMT96 Protocols
Résumé
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 1997 to 2008, 194 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 [1–45] 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.