Originally published as JCO Early Release 10.1200/JCO.2008.19.3367 on May 11 2009

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

From the Department of Pediatric Hematology/Oncology, Children's University Hospital, Giessen; Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, Kiel; Department of Pediatric Hematology/Oncology, Children's University Hospital, Tübingen; Department of Pediatric Hematology/Oncology, Children's University Hospital, Aachen; Department of Pediatric Hematology/Oncology, Children's Hospital, Sankt Augustin; Department of Pediatric Hematology/Oncology, Children's University Hospital, Göttingen; and Department of Pediatric Hematology/Oncology, Charité Universitätsmedizin Berlin, Berlin, Germany.

Corresponding author: Birgit Burkhardt, MD, PhD, Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller Str 3 Haus 9, 24105 Kiel, Germany; e-mail: birgit.burkhardt{at}uk-sh.de.

Purpose Little is known about the outcome of pediatric patients with lymphoblastic lymphoma (LBL) who suffer from progressive disease or relapse.

Patients and Methods We analyzed the pattern of LBL relapses after current non-Hodgkin's lymphoma Berlin-Frankfurt-Muenster (BFM) frontline therapy between April 1990 and March 2003. Relapse therapy was according to acute lymphoblastic leukemia (ALL) –Relapse-BFM protocols or ALL-BFM protocols for high-risk patients.

Results Twenty-eight (11%) of 251 registered patients with precursor T-cell LBL (T-LBL) and six (8%) of 73 patients with precursor B-cell LBL (pB-LBL) suffered from relapse. Of the 28 patients with T-LBL, one died from infection during relapse chemotherapy, 18 failed to achieve stable remission and died from disease progression, and nine reached allogeneic stem-cell transplantation (SCT). Two of these nine patients who underwent SCT died from transplantation-associated toxicity, three died from disease progression, and four are still alive. These four patients are in second remission of their lymphoma for 48, 68, 125, and 131 months, respectively, after allogeneic SCT. One of the four patients developed colon adenocarcinoma 47 months after SCT. Of the six patients with pB-LBL who experienced relapse, one patient died as a result of toxicity of relapse chemotherapy, two died from disease progression after chemotherapy, and three received allogeneic SCT. Of these, two died from subsequent disease progression, and one is still alive 57 months after allogeneic SCT.

Conclusion 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.

Presented in part at the Second International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin's Lymphoma, May 18-20, 2006, New York, NY; and the 50th American Society of Hematology Annual Meeting, December 6-9, 2008, San Francisco, CA.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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