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Published on: February 21, 2021
Pediatric myeloid/NK cell precursor lymphoma/leukemia expressing T/NK immunophenotype markers
Yoshiko Hashii1, Tokuko Okuda, Hideaki Ohta
1Department of Developmental Medicine (Pediatrics), Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan. yhashii@ped.med.osaka-u.ac.jp
International Journal of Hematology
|February 11, 2010
Summary
This case study describes a rare myeloid/NK cell precursor leukemia in a child, highlighting its unique immunophenotype and refractory nature. The findings suggest a common precursor for both myeloid and NK cell malignancies.
Area of Science:
- Hematology
- Immunophenotyping
- Oncology
Background:
- Myeloid/NK cell precursor lymphoma/leukemia is a rare hematologic malignancy.
- Its precise cell of origin remains debated, with suggestions of precursor NK cell origin.
Observation:
- A 1-year-old boy presented with a skin nodule indicative of myeloid/NK cell precursor lymphoma/leukemia.
- Initial biopsy showed a CD56(+), myeloid antigen(+), CD7(-), CD34(-) immunophenotype.
- Recurrent disease exhibited a shift to CD3(+) and TCRgammadelta(+) expression, with loss of MPO and TdT.
Findings:
- The patient's blast cells lacked typical CD34 and CD7 expression but expressed myeloid antigens, differing from classical myeloid/NK cell precursor acute leukemia.
- Predominant CD94 1A transcript expression suggested an immature NK cell origin.
- Acquired CD3 expression and TCRgammadelta rearrangement at recurrence indicated clonal evolution.
Implications:
- This case presents overlapping features of myeloid/NK precursor acute leukemia and blastic NK/precursor acute lymphoma/leukemia.
- The findings suggest the possibility of two subclones originating from a common precursor.
- Understanding these complex immunophenotypic shifts is crucial for diagnosing and managing rare hematologic malignancies.
