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Introduction: Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) is a high risk form of ALL associated with dismal outcomes in the pre-tyrosine kinase inhibitor (TKI) era. Addition of a TKI to chemotherapy improves outcomes. Therefore, testing for the presence of the Philadelphia chromosome by at least two methods at the time of diagnosis is critical. Diagnostic testing may include karyotype, fluorescent in situ hybridisation (FISH), and RT-PCR for the BCR::ABL1 transcript. The significance of low-level BCR::ABL1 transcript by RT-PCR in the absence of the Philadelphia chromosome on karyotype or by FISH is unknown., Methods: This is a retrospective review of children diagnosed with acute leukemia at our institution from 2010 to 2020. Those positive for the BCR::ABL1 transcript by qualitative RT-PCR, and negative for t(9;22) by karyotype or FISH were analyzed for demographics, cytogenetic and molecular features at diagnosis and relapse, treatment and outcomes. The Kaplan-Meier method was used to estimate event-free and overall survival., Results: Forty-seven of 306 (15%) patients with Ph- ALL had low-level BCR::ABL1 detected by RT-PCR. Most (77%) had B-cell ALL. The e1a2 transcript was detected most frequently, in 43 (91%) patients. BCR::ABL1 was quantifiable in 12/43 (28%) patients, with a median of 0.0008% (range 0.0003-0.095%). Seven patients (15%) relapsed. No patient with low-level BCR::ABL1 at diagnosis developed Ph + ALL at relapse. There was no difference in 5-year event-free (77% versus 81%, p = 0.407) or overall survival (86% versus 91%, p = 0.3) between children with low-level BCR::ABL1 (n = 47) and those without (n = 259)., Conclusion: BCR::ABL1 low-level positivity in children with newly diagnosed Ph- ALL is a relatively common finding and did not adversely affect outcome for patients treated using a contemporary risk-adapted approach., (© 2024 Wiley Periodicals LLC.)

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare and distinct subtype of peripheral T-cell lymphoma, representing <1% of all non-Hodgkin lymphomas. SPTCL usually arises in the fourth decade of life with multifocal involvement of the limbs and trunk. Orbital disease is uncommon. We present the youngest known case of orbital SPTCL in a 3-year-old child, where the diagnosis was initially confounded by a lower eyelid mass masquerading as preseptal cellulitis. MRI revealed a poorly defined anterior orbital mass. Immunophenotyping and histological analysis of an orbital biopsy specimen confirmed SPTCL, which was managed by the pediatric oncology team with multiagent chemotherapy. This case is unique due to the young age of presentation and primary orbital involvement. Nonresolving or atypical periorbital cellulitis needs to be investigated, as malignancy can mimic such conditions., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)