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Leukostasis in Children and Adolescents with Chronic Myeloid Leukemia: Japanese Pediatric Leukemia/Lymphoma Study
Hidemitsu Kurosawa1, Akihiko Tanizawa2, Chikako Tono3
1Department of Pediatrics, Dokkyo Medical University School of Medicine, Tochigi, Japan.
Insights
Leukostasis in pediatric chronic myeloid leukemia (CML) is characterized by high white blood cell counts and enlarged spleens. Symptoms like ocular issues and priapism were observed, often resolving with imatinib treatment.
Area of Science:
- Pediatric Hematology
- Oncology
- Leukemia Research
Background:
- Leukostasis in pediatric chronic myeloid leukemia (CML) remains poorly understood.
- This study aimed to define the characteristics of leukostasis in young CML patients.
Purpose of the Study:
- To determine the clinical features and outcomes of leukostasis in children and adolescents with CML.
- To provide insights into the presentation and management of this complication.
Main Methods:
- Retrospective analysis of 238 pediatric CML cases from the Japanese Pediatric Leukemia/Lymphoma Study Group (1996-2011).
- Evaluation of patient data for leukostasis diagnosis, clinical symptoms, and treatment response.
Main Results:
- Leukostasis was identified in 9.7% of pediatric CML patients.
- Patients with leukostasis exhibited significantly higher leukocyte counts and larger spleen sizes.
- Common symptoms included ocular manifestations (14 cases) and priapism (4 cases); most cases resolved with imatinib therapy.
Conclusions:
- This study presents the largest cohort of pediatric and adolescent CML patients with reported leukostasis.
- Findings offer valuable insights into the characteristics and recent trends of leukostasis in this demographic.
Background:
The details of leukostasis in children and adolescents with chronic myeloid leukemia (CML) are unknown. This study determined the characteristics of leukostasis in children and adolescents with CML.
Procedure:
A total of 256 cases from a retrospective study of patients with CML conducted by the Japanese Pediatric Leukemia/Lymphoma Study Group from 1996 to 2011 were analyzed, and of these, 238 cases were evaluated in this study.
Results:
Leukostasis was diagnosed in 23 patients (9.7%). The median leukocyte count and spleen size below the left costal margin in cases with leukostasis were significantly higher and larger when compared to those in cases without leukostasis (458.5 × 10(9) /l vs. 151.8 × 10(9) /l (P < 0.01), and 13 vs. 5 cm (P < 0.01), respectively). Leukostasis occurred with ocular symptoms in 14 cases, priapism in four cases, and dyspnea, syncope, headache, knee pain, difficulty hearing, and aseptic necrosis of the femoral head in one case each. One case had two leukostasis symptoms simultaneously. Three cases were diagnosed before imatinib became available. Five cases received special treatment, and in the remaining 15 cases, all of these symptoms resolved after treatment with imatinib.
Conclusions:
This retrospective study represents the largest series of children and adolescents in which leukostasis of CML has been reported. Our data provide useful insight into the characteristics of leukostasis in recent cases of children and adolescents with CML.