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Blood, Vol. 95 No. 2 (January 15), 2000:
pp. 416-421
Intensive ALL-type therapy without local radiotherapy provides a
90% event-free survival for children with T-cell lymphoblastic
lymphoma: a BFM Group report
Alfred Reiter,
Martin Schrappe,
Wolf-Dieter Ludwig,
Markus Tiemann,
Reza Parwaresch,
Martin Zimmermann,
Eckard Schirg,
Günter Henze,
Günther Schellong,
Helmut Gadner, and
Hansjörg Riehm on behalf of
the Berlin-Frankfurt-Münster Group
From the Department of Pediatric Hematology and Oncology,
Medizinische Hochschule, D-30625 Hannover, Germany; Department of
Hematology, Oncology, and Tumor Immunology, Charité, Humboldt
Universität Berlin, D-13122 Berlin, Germany; Lymphnode
Registry of The Society of German Pathologists, Institute of
Hematopathology, Christian-Albrechts-Universität, D-24105 Kiel,
Germany; Department of Diagnostic Radiology, Medizinische Hochschule,
D-30625 Hannover, Germany; Department of Pediatric Hematology and
Oncology, Charité, Humboldt University, D-13353 Berlin, Germany;
Department of Pediatric Hematology and Oncology, Westfälische
Wilhelms-Universität, D-48129 Münster, Germany; and St.
Anna Kinderspital, A-1090 Vienna, Austria.
The purpose of our study was to investigate the efficacy of an acute
lymphoblastic leukemia (ALL)-type treatment with moderate-dose, prophylactic cranial irradiation and without local radiotherapy for
childhood T-cell lymphoblastic lymphoma (T-LBL). From April 1990 to
March 1995, 105 evaluable patients, 1.1 to 16.4 years of age, with
T-LBL were enrolled in study NHL-BFM 90 (non-Hodgkin's lymphoma-Berlin-Frankfurt-Munster 90). They received an 8-drug induction over 9 weeks followed by an 8-week consolidation including methotrexate (MTX) 5 g/m2 × 4. Patients with stage I
(n = 2) and II (n = 2) continued with maintenance therapy
(6-mercaptopurine daily and MTX weekly, both orally) until a total
therapy duration of 24 months. Patients with stage III (n = 82) and
IV (n = 19) received an 8-drug intensification over 7 weeks and
cranial radiotherapy (12 Gy for prophylaxis) after consolidation,
followed by maintenance. Residual tumor after induction had to be
resected. Patients received intensified chemotherapy if tumor
regression on day 33 of induction was less than 70% or when vital
residual tumor was present after the complete induction phase. With a
median follow-up of 4.5 years, the estimated event-free survival at 5 years is 90% (95% confidence interval, 82%-100%). Events were 1 early death, 8 tumor failures, and 1 secondary acute myeloid
leukemia. A total of 101 patients were evaluable for the speed of tumor response. Two patients received intensified therapy due
to less than 70% tumor regression on day 33. Of 19 patients with tumor
residues after induction, 2 relapsed as compared to 4 of 80 patients
with complete tumor regression. We conclude that, with intensive
ALL-type chemotherapy including moderate cumulative doses of
anthracyclines 240 mg/m2 and cyclophosphamide (3 g/m2) and moderate-dose prophylactic cranial irradiation
but no local radiotherapy, an event-free survival rate of 90%
can be achieved in childhood T-LBL.
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