Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®): Treatment - Health Professional Information
Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®): Treatment - Health Professional Information [NCI]-Treatment of Relapsed Childhood ALL
Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®): Treatment - Health Professional Information [NCI] Guide
Children with Down syndrome with relapse of ALL had inferior outcomes as reported in a BFM report before 2000, primarily due to increased induction deaths and treatment-related mortality. However, since 2000, with improvements in supportive care, there have been no differences in outcome between patients with and without Down syndrome.[19]
Risk group classification at initial diagnosis
The COG reported that risk group classification at the time of initial diagnosis was prognostically significant after relapse; patients who met National Cancer Institute (NCI) standard-risk criteria at initial diagnosis fared better after relapse than did NCI high-risk patients.[13]
Response to reinduction therapy
Patients with marrow relapses who have persistent morphologic disease at the end of the first month of reinduction therapy have an extremely poor prognosis, even if they subsequently achieve a second complete remission (CR2).[20][Level of evidence: 2Di]; [21][Level of evidence: 3iiiA] Several studies have demonstrated that minimal residual disease (MRD) levels after the achievement of CR2 are of prognostic significance in relapsed ALL.[20,22,23,24]; [25][Level of evidence: 3iiiDi] High levels of MRD at the end of reinduction and at later time points have been correlated with an extremely high risk of subsequent relapse.
Cytogenetics/genomic alterations
TP53 alterations (mutations and/or copy number alterations) are observed in approximately 11% of patients with ALL at first relapse and have been associated with an increased likelihood of persistent leukemia after initial reinduction (38.5% TP53 alteration vs. 12.5% TP53 wild-type) and poor event-free survival (EFS) (9% TP53 alteration vs. 49% TP53 wild-type). Approximately one-half of the TP53 alterations were present at initial diagnosis and half were newly observed at time of relapse.[26] A second genomic alteration found to predict for poor prognosis in patients with B-precursor ALL in first bone marrow relapse is IKZF1 deletion.[27] The frequency of IKZF1 deletion in B-precursor ALL patients at first relapse patients was 33% in patients in the Acute Lymphoblastic Leukemia Relapse (ALL-REZ) BFM 2002 study, which was approximately twice as high as the frequency described in children at initial diagnosis of ALL.[27]
Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Relapsed Childhood ALL
Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®): Treatment - Health Professional Information [NCI] Guide
- General Information About Childhood Acute Lymphoblastic Leukemia (ALL)
- Risk-based Treatment Assignment
- Treatment Option Overview for Childhood ALL
- Treatment for Newly Diagnosed Childhood ALL
- Postinduction Treatment for Childhood ALL
- CNS-directed Therapy for Childhood ALL
- Postinduction Treatment for Specific ALL Subgroups
- Treatment of Relapsed Childhood ALL
- Changes to this Summary (05 / 02 / 2014)
- About This PDQ Summary
- Get More Information From NCI
Children with Down syndrome with relapse of ALL had inferior outcomes as reported in a BFM report before 2000, primarily due to increased induction deaths and treatment-related mortality. However, since 2000, with improvements in supportive care, there have been no differences in outcome between patients with and without Down syndrome.[19]
Risk group classification at initial diagnosis
The COG reported that risk group classification at the time of initial diagnosis was prognostically significant after relapse; patients who met National Cancer Institute (NCI) standard-risk criteria at initial diagnosis fared better after relapse than did NCI high-risk patients.[13]
Response to reinduction therapy
Patients with marrow relapses who have persistent morphologic disease at the end of the first month of reinduction therapy have an extremely poor prognosis, even if they subsequently achieve a second complete remission (CR2).[20][Level of evidence: 2Di]; [21][Level of evidence: 3iiiA] Several studies have demonstrated that minimal residual disease (MRD) levels after the achievement of CR2 are of prognostic significance in relapsed ALL.[20,22,23,24]; [25][Level of evidence: 3iiiDi] High levels of MRD at the end of reinduction and at later time points have been correlated with an extremely high risk of subsequent relapse.
Cytogenetics/genomic alterations
TP53 alterations (mutations and/or copy number alterations) are observed in approximately 11% of patients with ALL at first relapse and have been associated with an increased likelihood of persistent leukemia after initial reinduction (38.5% TP53 alteration vs. 12.5% TP53 wild-type) and poor event-free survival (EFS) (9% TP53 alteration vs. 49% TP53 wild-type). Approximately one-half of the TP53 alterations were present at initial diagnosis and half were newly observed at time of relapse.[26] A second genomic alteration found to predict for poor prognosis in patients with B-precursor ALL in first bone marrow relapse is IKZF1 deletion.[27] The frequency of IKZF1 deletion in B-precursor ALL patients at first relapse patients was 33% in patients in the Acute Lymphoblastic Leukemia Relapse (ALL-REZ) BFM 2002 study, which was approximately twice as high as the frequency described in children at initial diagnosis of ALL.[27]
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