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Acute lymphoblastic leukemia with coexpression of CD56 and CD57: case report
Kousaku Matsubara1, Kazuo Yura, Takuya Hirata
1Department of Pediatrics, Nishi-Kobe Medical Center, 5-7-1 Kojidai, Nishi-ku, Kobe 651-2273, Japan. kskmatsu@s4.dion.ne.jp
Insights
This study details a rare pediatric case of acute lymphoblastic leukemia (ALL) in a 6-year-old girl. The leukemia cells uniquely expressed markers of both B-cell precursors and natural killer cells, a finding previously undocumented in pediatric B-lineage ALL.
Area of Science:
- Pediatric Hematology Oncology
- Immunophenotyping
- Molecular Genetics
Background:
- Acute lymphoblastic leukemia (ALL) is a heterogeneous malignancy with diverse immunophenotypes.
- Understanding immunophenotypic variations is crucial for accurate diagnosis and risk stratification in pediatric ALL.
- B-lineage ALL typically exhibits specific B-cell precursor markers.
Observation:
- A 6-year-old girl presented with massive hepatosplenomegaly and acute lymphoblastic leukemia (ALL).
- Leukemic cells were myeloperoxidase-negative, morphologically lymphoblastic, and positive for B-precursor antigens (CD10, CD19).
- Unusually, these B-lineage cells also expressed natural killer cell markers (CD56, CD57).
Findings:
- Genetic analysis revealed rearrangements in immunoglobulin heavy chain alleles and T-cell receptor (TCR)-beta and -delta genes, supporting a B-precursor origin.
- The coexpression of CD56 and CD57 on B-lineage ALL cells is a novel observation in pediatric oncology.
- This immunophenotype presents a diagnostic challenge, bridging B-cell and natural killer cell lineages.
Implications:
- This case expands the known spectrum of immunophenotypic diversity in pediatric ALL.
- The unique immunophenotype may necessitate tailored treatment strategies for high-risk ALL.
- Further research into such mixed-lineage leukemias is warranted to elucidate their biology and optimize therapeutic approaches.
Abstract:
The authors present the clinical profile of a 6-year-old girl with an unusual immunophenotype of acute lymphoblastic leukemia (ALL). At the initial presentation, massive hepatosplenomegaly developed. The leukemic cells were myeloperoxidase-negative and morphologically lymphoblastic. These cells were positive for B-precursor-cell (CD10, CD19) antigens and natural killer cells (CD56, CD57). Rearrangements of both immunoglobulin heavy chain alleles and monoallelic rearrangement of T-cell receptors (TCRs)-beta and -delta genes, but not that of TCR-gamma gene, were detected, suggesting that these cells being of B-precursor origin. The patient received chemotherapy for extremely high-risk ALL with a good response. To the authors' knowledge, this is the first pediatric case describing coexpression of CD56 and CD57 on B-lineage ALL.
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