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Juvenile chronic myeloid leukemia. A malignancy of S-100 protein-positive histiocytes
Insights
Juvenile chronic myeloid leukemia (JCML) is a rare histiocytic malignancy in children. S-100 protein is identified as a useful diagnostic marker for JCML.
Area of Science:
- Hematology
- Pediatric Oncology
- Pathology
Background:
- Juvenile chronic myeloid leukemia (JCML) is a rare myeloproliferative neoplasm affecting young children.
- JCML presents with distinct clinical and hematological features, often differing from adult CML.
Purpose of the Study:
- To report three cases of JCML, detailing their clinical presentation, pathological findings, and immunophenotype.
- To investigate the cellular origin and diagnostic markers of JCML.
Main Methods:
- Clinical case reporting and analysis.
- Bone marrow morphology and histopathology examination.
- Immunophenotypic analysis of neoplastic cells.
Main Results:
- Patients presented with lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, and elevated white blood cell counts.
- Philadelphia chromosome was absent in most cases.
- Neoplastic cells exhibited features of dendritic cells and mononuclear phagocytes, with S-100 protein positivity.
Conclusions:
- JCML represents a unique histiocytic malignancy.
- S-100 protein is a valuable marker for diagnosing JCML.
- Understanding the immunophenotype aids in classifying JCML within histiocytic disorders.
Abstract:
Three cases of juvenile chronic myeloid leukemia (JCML) are reported. The patients were aged 3-4.5 years and presented with generalized lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, elevated white blood cell count with monocytosis, and high fetal hemoglobin level. Philadelphia chromosome was absent in two cases studied. The bone marrow showed myeloid hyperplasia with increased monocytoid cells and blasts. Biopsy or postmortem material available in two cases revealed malignant infiltration of lymph nodes, liver, spleen, lungs, intestines, and skin. The neoplastic cells ranged from cells with irregular nuclei possessing nuclear grooves to large blastic cells with round to lobulated nuclei and prominent nucleoli. They showed weak staining for acid phosphatase and nonspecific esterase and exhibited the immunophenotype EBM11+KiM1+KiM6+KiM8+CD4+HLADR+ S-100 protein+. The neoplastic cells of JCML therefore share features of dendritic cells and mononuclear phagocytes. The authors' findings show that JCML is a unique histiocytic malignancy in which S-100 protein is a useful marker.