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Blood, 15 June 2001, Vol. 97, No. 12, pp. 3699-3706
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is
efficacious treatment for pediatric anaplastic large cell lymphoma: a
report of the Berlin-Frankfurt-Münster Group Trial
NHL-BFM 90
Kathrin Seidemann,
Markus Tiemann,
Martin Schrappe,
Elif Yakisan,
Ingrid Simonitsch,
Gritta Janka-Schaub,
Wolfgang Dörffel,
Martin Zimmermann,
Georg Mann,
Helmut Gadner,
Reza Parwaresch,
Hansjörg Riehm, and
Alfred Reiter
From the Department of Pediatric Hematology and
Oncology, Medizinische Hochschule, Hannover; Lymphnode Registry Kiel
founded by the Society of German Pathologists, Institute of
Hematopathology, Christian-Albrechts-University, Kiel; the
Department of Pediatric Hematology and Oncology, University of Hamburg;
the Second Department of Pediatrics, Klinikum Buch, Berlin; and the
Department of Pediatric Hematology and Oncology,
Justus-Liebig-University, Giessen, Germany; and the Institute of
Pathology, University of Vienna and St Anna Children's
Hospital, Vienna, Austria.
Anaplastic large-cell lymphoma (ALCL) accounts for approximately
10% of pediatric non-Hodgkin lymphoma (NHL). Previous experience from
NHL-Berlin-Frankfurt-Münster (BFM) trials indicated that the
short-pulse B-NHL-type treatment strategy may also be efficacious for
ALCL. The purpose of this study was to test the efficacy of this
protocol for treatment of childhood ALCL in a large prospective multicenter trial and to define risk factors. From April 1990 to March
1995, 89 patients younger than 18 years of age with newly diagnosed
ALCL were enrolled in trial NHL-BFM 90. Immunophenotype was T-cell in
40 patients, B-cell in 5, null in 31, and not determined in 13. Stages
were as follows: I, n = 8; II, n = 20; III, n = 55; IV,
n = 6. Extranodal manifestations were as follows: mediastinum, n = 28; lung, n = 13; skin, n = 16; soft tissue, n = 13; bone, n = 14; central nervous system, n = 1; bone marrow, n = 5. After a cytoreductive prephase, treatment was stratified into 3 branches: patients in K1 (stage I and II resected) received three 5-day courses (methotrexate [MTX] 0.5 g/m2,
dexamethasone, oxazaphorins, etoposide, cytarabine,
doxorubicin, and intrathecal therapy); patients in K2 (stage II
nonresected and stage III) received 6 courses; patients in K3 (stage IV
or multifocal bone disease) received 6 intensified courses including MTX 5 g/m2, high-dose cytarabine/etoposide. The
Kaplan-Meier estimate for a 5-year event-free survival was
76% ± 5% (median follow-up, 5.6 years) for all patients and 100%,
73% ± 6%, and 79% ± 11% for K1, K2, and K3, respectively.
Events were as follows: progression during therapy, n = 2;
progression or relapse after therapy, n = 20; second
malignancy, n = 1. It was concluded that short-pulse chemotherapy,
stratified according to stage, is effective treatment for pediatric
ALCL. B symptoms were associated with increased risk of failure.
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