Publications by authors named "S Alberti-Violetti"
Rare subtypes of cutaneous T-cell lymphomas (CTCL) include four entities, primary cutaneous γδ T-cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, and primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders, primary cutaneous acral CD8+ T-cell lymphoma, which were previously considered provisional and are now included in the new 5 World Health Organization classification of hematolymphoid tumors as distinct entities. An updated summary of the clinical, histological, and genomic characteristics of these uncommon CTCL subtypes is given in this review, with a focus on the growing body of knowledge regarding their classification and possible treatment strategies.
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- This text discusses two patients diagnosed with epidermotropic T-cell lymphoma that exhibited characteristics similar to mycosis fungoides (MF) and expressed ALK, prompting an analysis of ALK+ primary cutaneous T-cell lymphomas (pcTCL).
- It highlights the unique histological features of these MF-like cases, which include specific cell types and expressions of certain markers, and emphasizes the need for careful investigation for ALK expression.
- The text also addresses the rarity of bona fide ALK+ pcTCL, the challenges in diagnosis due to potential misinterpretation, and the lack of conclusive data on treatment strategies, suggesting that MF-like management may be used while considering targeted therapies in advanced cases.
Article Synopsis
- Primary cutaneous lymphomas are skin-specific immune system tumors that typically do not show other symptoms outside the skin at diagnosis; research on these conditions in children and adolescents is limited.
- This study aimed to fill the knowledge gap by examining the clinical, histological, and molecular features of pediatric patients with primary cutaneous lymphoma, resulting in the creation of the Paediatric Primary Cutaneous Lymphoma Atlas to assist in diagnosis.
- The retrospective analysis included 101 patients, with lymphomatoid papulosis and mycosis fungoides being the most common subtypes; long-term follow-up indicated some patients face significant health challenges, with three deaths recorded due to the condition.
Article Synopsis
- Lymphomatoid papulosis (LyP) is a rare skin disorder affecting T-cells, and there's limited information about its impact on children.
- A study analyzed data from 87 pediatric patients diagnosed with LyP over a 24-year period, noting that most cases were initially misdiagnosed and the average age at onset was 7 years.
- The prognosis appears positive, with a 100% overall survival rate, although 10% of patients had associated blood cancers, indicating a significantly higher risk of malignancy compared to the general youth population.