Outcome of children with high-risk acute lymphoblastic leukemia (HR-ALL): Nordic results on an intensive regimen with restricted central nervous system irradiation

• BACKGROUND: Improvement in outcome of childhood high-risk (HR) ALL was sought with a very intensive Nordic protocol leaving most patients without CNS-RT. METHODS: A total of 426 consecutive children entered the NOPHO-92 HR-ALL program. HR criteria included WBC > or =50 x 10(9)/L, CNS or testicular involvement, T-cell, lymphomatous features, t(9;22), t(4;11), or slow response. Of these, 152 children had very high risk (VHR) with special definitions. CNS consolidation was based on high-dose MTX (8 g/m2) and ARA-C (12 g/m2) alternating. VHR patients also received cranial RT. RESULTS: The 9-year EFS was 61 +/- 3%, OS 74 +/- 2%, and EFS for T-ALL 62 +/- 4%. Cumulative incidence of isolated CNS relapse was 4.7 +/- 1%, and CNS relapse in total 9.9 +/- 2%. Poor prognostic factors were WBC > or =200 x 10(9)/L and a very slow response. CONCLUSIONS: HR-ALL was successfully treated on the NOPHO-92 regimen, with a relatively low CNS relapse rate for non-irradiated children. WBC > or =200 x 10(9)/L and very slow response emerged as strong poor prognostic factors.

Nyckelord

Adolescent

dosage/*therapeutic use

Central Nervous System Neoplasms/*therapy

Child

Child

Preschool

Cohort Studies

Combined Modality Therapy

*Cranial Irradiation

Cytarabine/administration & dosage

Disease-Free Survival

Female

Humans

Infant

Leukemia

Lymphocytic

Acute/*therapy

Male

Methotrexate/administration & dosage

Risk Factors

Stem Cell Transplantation

Transplantation

Homologous

Treatment Outcome

Publikations- och innehållstyp

ref (ämneskategori)

art (ämneskategori)