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Published on: October 14, 2016
Myeloid sarcoma in a one-month-old infant
Chihiro Wakusawa1, Taku Fujimura, Akira Hashimoto
1Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Insights
Congenital myeloid sarcoma, a rare leukemia manifestation, presented as a skin lesion in an infant without systemic leukemia. Prompt chemotherapy resolved the lesion, with no recurrence for 1.5 years.
Area of Science:
- Hematology
- Pediatric Oncology
- Dermatology
Background:
- Myeloid sarcoma is a rare extramedullary tumor of myeloid blasts.
- It can be associated with systemic leukemia or occur in isolation.
- Congenital myeloid sarcoma is exceptionally rare, particularly without overt signs of leukemia.
Observation:
- A 1-month-old infant presented with a bruise-like skin plaque.
- Histopathology revealed atypical large lymphocytes (CD4+, CD56-, CD68+) in the dermis and subcutaneous tissue.
- Bone marrow biopsy showed minimal blast cells (3.4%) with mixed-lineage leukemia gene abnormality.
Findings:
- The infant was diagnosed with myeloid sarcoma not meeting criteria for systemic leukemia.
- Skin lesions resolved within 10 days of chemotherapy.
- No recurrence was observed for 1.5 years post-treatment.
Implications:
- This case highlights congenital myeloid sarcoma as a distinct entity presenting primarily with cutaneous manifestations.
- Early diagnosis and treatment can lead to favorable outcomes, even in the absence of systemic leukemia.
- Further research into the unique biology and treatment of isolated congenital myeloid sarcoma is warranted.
Abstract:
Myeloid sarcoma, which sometimes accompanies recurrent systemic leukemia, is a rare manifestation of congenital leukemia. We describe the case of a 1-month-old Japanese girl with myeloid sarcoma, who did not fulfill the criteria of systemic leukemia. The physical examination revealed a bruise-like plaque on the right side of her chest. Histopathologically, there were CD4+ CD56- CD68+ atypical large lymphocytes densely infiltrating the upper dermis and the subcutaneous tissue. Biopsy specimens from bone marrow contained only 3.4% of blast cells, showing an abnormality of the mixed-lineage leukemia gene. The skin lesions disappeared 10 days after chemotherapy, and there was no recurrence for one and a half years.
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