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Extramedullary relapse in childhood leukemia.
1First Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan.
Acta Paediatrica Japonica : Overseas Edition
|August 1, 1991
Summary
Extramedullary leukemia, particularly CNS leukemia, is common in childhood leukemia survivors. Survival rates vary by leukemia type and relapse site, with some forms having poor prognoses.
Area of Science:
- Pediatric Oncology
- Hematology
- Cancer Epidemiology
Background:
- Increasing long-term survival in childhood leukemia necessitates focus on extramedullary leukemia prophylaxis.
- Extramedullary leukemia (EML) is a significant concern in pediatric leukemia treatment.
Purpose of the Study:
- To investigate the patterns and outcomes of extramedullary leukemia in children.
- To analyze the incidence and common sites of EML in acute lymphoblastic leukemia (ALL) and acute non-lymphoblastic leukemia (ANLL).
Main Methods:
- Retrospective review of 2,317 childhood leukemia patients (≤15 years) treated between 1976-1985.
- Data collected from 38 institutes across Japan.
- Analysis of EML occurrence, sites, and survival rates based on leukemia type and relapse patterns.
Main Results:
- EML occurred in 22.4% of ALL patients and 16.3% of ANLL patients.
- Central Nervous System (CNS) leukemia was the most frequent EML site in both ALL (81.6%) and ANLL (71.4%).
- Testicular leukemia (23.0%) was common in ALL, while cutaneous leukemia (15.9%) was noted in ANLL. Survival rates for isolated CNS and testicular relapses were around 40%, but significantly lower with bone marrow relapse. Cutaneous and renal leukemia had fatal outcomes.
Conclusions:
- CNS leukemia is the predominant form of extramedullary leukemia in childhood leukemia.
- Prophylaxis and treatment strategies for EML, especially CNS and testicular leukemia, are crucial for improving outcomes.
- Certain EML sites like cutaneous and renal leukemia are associated with a grim prognosis.