CLASSIFICATION OF NON-HODGKINS-LYMPHOMA O F CHILDHOOD AND ADOLESCENCEAND STRATIFICATION OF TREATMENT MODALITIES - AN INTERIM ANALYSIS OF STUDY NHL-BFM-90 .1.
A. Reiter et al., CLASSIFICATION OF NON-HODGKINS-LYMPHOMA O F CHILDHOOD AND ADOLESCENCEAND STRATIFICATION OF TREATMENT MODALITIES - AN INTERIM ANALYSIS OF STUDY NHL-BFM-90 .1., Klinische Padiatrie, 206(4), 1994, pp. 222-233
One of the goals of the study NHL-BFM 90 was to investigate the distri
bution and prognosis of the different subtypes of Non-Hodgkin's Lympho
ma (NHL) in children and adolescents according to histological, cytomo
rphological and immunological characteristics. From 4/1990 to 12/1992,
346 patients (pts) (84 females, 262 males) were enrolled (median age:
9.1 years; range: 0,8-17, 9 years). Histology was available from 290
pts (84%), cytomorphology from 155 (44%), and immunophenotyping from 2
45 (70%). Cases with L1 oder L2 cytomorphology according to the French
-American-British Classification were classified as lymphoblastic lymp
homa and those with L3 cytomorphology as Burkitt-Type lymphoma or acut
e B-cell leukemia (B-ALL) if a histological classification was not ava
ilable. By means of the combined analysis of all three diagnostic crit
erias the classification of the NHL according to the updated Kiel-clas
sification was possible in 312 cases: 49% were classified as Burkitt-t
ype-lymphoma (incl. BALL), 22% als lymphoblastic lymphoma, 10% as larg
e cell anaplastic lymphoma (LCAL), 6% as centroblastic lymphoma, only
few cases were classified as NHL of other subtypes, 3 pts (1%) suffere
d from low grade malignant lymphomas, and in 34 pts (10%) the NHL was
not further classified. Patients were stratified according to NHL-sube
ntities in 3 branches (Non-B-NHL, B-NHL, LCAL) of different treatment
modalities. The estimated probability of a 3-year event free survival
(pEFS) was 88 +/- 2% for the whole group (follow up 7 to 40 months, me
dian 23 months) while pEFS of different subtypes was: lymphoblastic ly
mphoma: 91 +/- 4%; Burkitt-type-lymphoma/B-ALL: 90 +/- 2%; centroblast
ic lymphoma: 94 +/- 6%, LCAL: 88 +/- 6%. We conclude that the stratifi
cation of treatment modalities in study NHL-BFM 90 according to biolog
ical entities provided patients of different NHL-subtypes an equal cha
nce to survive event free. The efficacy of the treatment strategy for
rare subtypes, however, is not evaluable yet.