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An effective therapy for both undifferentiated (including Burkitt's)
lymphomas and lymphoblastic lymphomas in children and young adults
IT Magrath, C Janus, BK Edwards, R Spiegel, ES Jaffe, CW Berard, J Miliauskas, K Morris and R Barnwell
We have used a single intensive chemotherapy regimen in the treatment of
young patients with diffuse, aggressive, malignant lymphomas. There were
two major histologic types of lymphoma in our series: lymphoblastic
lymphomas, which presented most often with mediastinal tumor (64%), and
undifferentiated lymphomas (mostly Burkitt's lymphomas), which occurred
predominantly in the abdomen (86%). Our objective was to examine the
determinants of prognosis in a uniformly treated patient group that
included 31 children (2-16 yr) and 34 young adults 17-35 yr). Patients with
extensive bone marrow involvement (greater than 50% replacement by tumor
cells) were included in the study. Treatment consisted essentially of a
4-drug combination (cytoxan, adriamycin, vincristine, and prednisone)
alternating with a 42-hr methotrexate infusion, followed by leukovorin
rescue, and included intrathecal prophylactic therapy against central
nervous system (CNS) disease. Patients with localized or resected
undifferentiated lymphoma received 6 therapy cycles; all other patients
received 15 cycles. Radiation therapy was used only in exceptional
circumstances. Fifty-eight of 65 patients (89%) achieved complete
remission: 97% of children and 82% of adults. The estimated 3-yr survival
was 60% (SE 6.4%) with a median follow-up of 3 yr. Analysis of factors
associated with remission duration and survival indicated that bone marrow
involvement at referral and extensive disease were poor prognostic
variables. Patients with lymphoblastic lymphomas and patients with
completely resected undifferentiated lymphomas had the best prognosis (81%
+/- 12% and 94% +/- 6% estimated 3-yr survival, respectively). Patients
with extensive intraabdominal undifferentiated lymphoma (stage D) had the
worst prognosis (33% +/- 11% estimated 3 yr survival), but even in this
subgroup, bone marrow involvement was an adverse factor (estimated survival
in stage D patients with and without bone marrow involvement was 14% +/-
13% and 43% +/- 15%, respectively). Elevated uric acid and/or lactic
dehydrogenase (LDH) were also of prognostic significance, but predominantly
reflected state, i.e., extent of disease. Age did not significantly
influence prognosis. In the undifferentiated lymphoma subgroup, histology
(i.e., Burkitt's lymphoma versus non-Burkitt's lymphoma) was not of
prognostic significance. Total white count was below 1,000/cu mm in 39% of
cycles, and fever associated with granulocytopenia occurred in 17% of
cycles. Stomatitis of moderate to severe extent occurred in 50% of
cycles.(ABSTRACT TRUNCATED AT 400 WORDS)
Volume 63,
Issue 5,
pp. 1102-1111,
05/01/1984
Copyright © 1984 by The American Society of Hematology
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