Clin Pediatr Hematol Oncol.  2008 Oct;15(2):101-110.

Clinical Manifestations and Treatment Outcome of Chronic Myeloid Leukemia in Children and Adolescents

• Bae SY
• Han DK
• Baek HJ
• You JH
• Park EY
• Cho YK
• Kook H
• Hwang TJ

Abstract

PURPOSE: Chronic myeloid leukemia (CML) comprises 2~3% of childhood leukemia. The purpose of this study was to determine the main presenting features and to explore the feasibility and efficacy of imatinib mesylate and hematopoietic stem cell transplantation (SCT) in pediatric CML.
METHODS
We retrospectively evaluated 9 children with CML (chronic phase, 8; blastic crisis, 1) who were treated at the Chonnam National University Hospital between Jan. 1995 and Dec. 2007.
RESULTS
There were 4 boys and 5 girls, with a median age of 11.2 years (range, 1.8~16.5 years). An incidental diagnosis of CML was made in 3 children. The main presenting symptoms were abdominal discomfort, asthenia, and weight loss. Splenomegaly was found in 66.7%. Marked leukocytosis (median, 192,700/microliter) and anemia were common. Myeloid blastic crisis developed in 2 patients. Among 7 patients who received imatinib treatment, complete hematological remission was achieved in 6, and major molecular remission in 4. Myalgia, ostealgia, and mild bone marrow suppression were observed. Seven patients underwent SCTs with a median time interval from the time of diagnosis being 7 months. The 5-year estimated event free survival (EFS) rate is 69% with the median follow-up of 32 months after SCT.
CONCLUSION
The clinical and hematological findings of pediatric CML appeared to be similar to those in adults. Imatinib therapy seemed to be effective and well tolerated in children with CML. Allogeneic SCT may offer a cure in pediatric CML. Multicenter, nation-wide studies are warranted to establish the optimal treatment strategies in childhood CML.

Keyword

Chronic myeloid leukemia; Imatinib; Stem cell transplantation

MeSH Terms

Adolescent
Adult
Anemia
Asthenia
Benzamides
Bone Marrow
Child
Disease-Free Survival
Follow-Up Studies
Hematopoietic Stem Cell Transplantation
Humans
Leukemia
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Leukocytosis
Mesylates
Piperazines
Pyrimidines
Retrospective Studies
Splenomegaly
Stem Cell Transplantation
Treatment Outcome
Weight Loss
Imatinib Mesylate
Benzamides
Mesylates
Piperazines
Pyrimidines