Childhood and adolescent large‐cell lymphoma (LCL): A review of the children's cancer group experience

Abstract

We reviewed the clinical characteristics, treatment, and outcome of 67 children with localized and 212 with disseminated large-cell lymphoma (LCL) treated during a 20-year period in 5 consecutive Children's Cancer Group (CCG) non-Hodgkin's lymphoma (NHL) trials. Clinical outcomes for patients treated on the four earlier studies with moderatedose chemotherapy administered over 12-18 months were compared with patients treated most recently with short, intensive therapy. Median age at diagnosis was 12 years (range: 0-19 years). Male to female ratio was 1.8:1.0. Five-year event-free survival (EFS) was 92% ± 3.3% and 50 ± 3.5% for patients with localized LCL and disseminated LCL, respectively. After adjustment for lactate dehydrogenase (LDH), age at diagnosis, and BM involvement, short and intensive therapy as delivered on the most recent study, CCG-5911, was associated with an improved outcome (P < 0.05) compared to the four previous studies. Elevated LDH (≥500 IU/L) at diagnosis and young age (<5 years) were both significant independent predictors of poorer long-term EFS (P < 0.05). Long-term survival after relapse or other treatment failure was only 31% ± 4.7%. In summary, more recent shorter and intense therapy appears to be associated with superior event-free survival for children and adolescents with disseminated LCL. Large numbers of patients treated with shorter and intense therapy are required to confirm these preliminary observations. Am.

Evaluation of children with lympadenopathy

“…In the study performed by Cairo et al (11), a LDH level above 1000 IU/L was determined to be an independent prognostic factor in lymphomas. In other studies, LDH level as a prognostic criterion in lymphoma was reported to be 500 IU/L or lower or higher than two-fold of the normal (11)(12)(13)(14). In our study, the mean LDH value was found to be 665 IU/L.…”

Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents

“…[1][2][3][4][5][6][7][8] About 90% of histologic subtypes of childhood de novo high-risk (BM with or without CNS) B-NHL are of the aggressive subtype, including mature B-cell acute lymphoblastic leukemia (B-ALL), Burkitt lymphoma (BL) and Burkitt-like (BLL) histology and to a lesser extent, an intermediate subtype, diffuse large B-cell lymphoma (DLBCL). [9][10][11] Despite an improvement in overall survival (S) with multiagent intensive chemotherapy, there is a significant incidence of serious morbidity, including grade III/IV mucositis (40%-70%), systemic infection (60%-80%), myelosuppression (80%-100%), prolonged hospitalization (median 10-14 days during each induction cycle), and a toxic death rate of 1%-2% in remission with potential long-term cardiac and gonadal toxicities and secondary malignancies.…”

Revised International Pediatric Non-Hodgkin Lymphoma Staging System

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14] Currently, localized or limited stage NHL (stage I to II) has an approximate 95% to 100% 5-year event-free survival (EFS) rate. Furthermore, the prognosis for children with advanced-stage disease (stage III to IV) has doubled from a 5-year EFS of approximately 40% 30 years ago to more than 80%.…”