Poor Outcome for Children and Adolescents With Progressive Disease or Relapse of Lymphoblastic Lymphoma: A Report From the Berlin-Frankfurt-Muenster Group

Abstract

Using modern conventional therapy in the frontline treatment of LBL, 10% of patients suffer from progressive disease or relapse. Because of the extremely poor reinduction success, the salvage rate for these patients is poor, with only a 14% (SE = 6%) overall survival. Long-term survival was only achieved in those few patients who were able to undergo an allogeneic SCT.

Salvage therapy with nelarabine, etoposide, and cyclophosphamide in relapsed/refractory paediatric T‐cell lymphoblastic leukaemia and lymphoma

“…The two patients with LL had only a partial response to the therapy. This is consistent with data on the increased difficulty of getting relapsed LL patients into a CR2 (Burkhardt et al, 2009). Although our numbers are small, the response rate appeared to be better than for AraG alone in a similar refractory population (Berg et al, 2005).…”

“…On a recent intensive re-induction platform only two of seven patients (29%) with early relapse (<18 months from diagnosis) of T-cell ALL obtained a CR2; whereas 43 of 63 pre-B cell ALL (68%) went into a second remission . Patients with relapsed T-cell lymphoblastic lymphoma (LL) also have poor CR2 and EFS rates (Burkhardt et al, 2009). The Berlin-Frankfurt-Münster group classifies T-cell relapses as higher risk than their B-lineage counterparts while the Children's Oncology Group (COG) does not separate T from B lineage at relapse, although T-cell immunophenotype was found to be unfavourable in recent COG retrospective reviews of relapse (Barrett et al, 1994;Gaynon et al, 1998).…”

Outcome of children and adolescents with lymphoblastic lymphoma

“…This finding implies that a more sensitive method for investigating neoplastic cells in the BM at diagnosis may contribute to a more appropriate risk indeed an extremely poor prognosis. 1 The probability of 5-year survival of 20 patients with de novo LBL treated in the same manner was almost 80%, similar to that achieved in major treatment centers (75 to 90%). 3 conclusion Our findings confirm a favorable prognosis for children with LBL treated with ALL-based therapies.…”

Absence of terminal deoxynucleotidyl transferase expression identifies a subset of high-risk adult T-lymphoblastic leukemia/lymphoma

“…4 Patients with relapsed T-ALL/LBL usually respond poorly to augmented salvage chemotherapy and allogeneic hematopoietic stem cell transplant. 4,6 The key to improve survival therefore is to identify patients at high risk for relapse at the time of initial diagnosis and to implement risk-adapted therapy.…”