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Hemato, Volume 6, Issue 3 (September 2025) – 3 articles

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16 pages, 1110 KiB  
Editorial
What Is Still Unclear or Unresolved in Classic Hodgkin Lymphoma Pathobiology, Diagnosis, and Treatment
by Antonino Carbone, Mohamed Nazem Alibrahim and Annunziata Gloghini
Hemato 2025, 6(3), 20; https://doi.org/10.3390/hemato6030020 - 4 Jul 2025
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Abstract
In recent decades, significant progress in medicine has improved the outcomes for Hodgkin lymphoma (HL), a malignancy affecting approximately 8570 new patients annually in the United States and causing around 910 deaths per year [...] Full article
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17 pages, 1275 KiB  
Article
Intrachromosomal Amplification of Chromosome 21 (iAMP21) Impacts Event-Free Survival but Not Overall Survival Among Pediatric Patients with Acute Lymphoblastic Leukemia: A Single-Center Experience Using an Asparaginase-Intensified Spanish Regimen
by María Hidalgo, Eduardo Ramos-Elbal, José Antonio Galián, Helios Martínez-Banaclocha, Mercedes Plaza, Victoria Martínez-Sánchez, Ana María Galera, Irene Jiménez, María Esther Llinares, Mar Bermúdez, Alfredo Minguela and José Luis Fuster
Hemato 2025, 6(3), 19; https://doi.org/10.3390/hemato6030019 - 27 Jun 2025
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Abstract
Background/Objectives: Intrachromosomal amplification of chromosome 21 (iAMP21) represents a rare and heterogeneous distinct cytogenetic subgroup of B-cell precursor acute lymphoblastic leukemia (ALL) initially associated with a poor prognosis. Treatment intensification with additional doses of methotrexate and asparaginase was associated with better treatment outcomes. [...] Read more.
Background/Objectives: Intrachromosomal amplification of chromosome 21 (iAMP21) represents a rare and heterogeneous distinct cytogenetic subgroup of B-cell precursor acute lymphoblastic leukemia (ALL) initially associated with a poor prognosis. Treatment intensification with additional doses of methotrexate and asparaginase was associated with better treatment outcomes. Methods: In this retrospective single-center study, we evaluated the impact of iAMP21 on treatment outcome in a cohort of pediatric patients treated with an intensified asparaginase regimen and describe the genomic landscape of four patients with iAMP21. Results: Four out of 89 patients > 1 year old were classified as iAMP21 positive. Five-year event-free survival (EFS) was inferior in the iAMP21-positive group: 25% versus 85.6% (p = 0.001). The cumulative incidence of relapse and treatment-related mortality were 50% vs. 9.9% and 0% vs. 2.38%, respectively, in the iAMP21-positive and non-iAMP21 groups (p = 0.02 and 0.76, respectively). These results did not translate into a significant difference in overall survival: 100% vs. 93.7% (p = 0.6). The presence of iAMP21 (HR 7.68, 95% CI 2.04–29.05; p = 0.002) and a measurable residual disease ≥1% after induction on day +33 (HR 8.82, 95% CI 2.6–29.91; p = 0.001) retained significant negative impact on EFS in multivariate analysis. Conclusions: We found an independent significant prognostic impact of iAMP21 on EFS among pediatric patients with ALL, and clinical presentation and early treatment response did not classify these patients as HR. Diverse genetic backgrounds among iAMP21-positive patients might influence the treatment response and outcome of this heterogeneous disease. Full article
(This article belongs to the Section Leukemias)
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7 pages, 4563 KiB  
Case Report
Diagnosis and Treatment of Langerhans Cell Sarcoma: A Case Report and Review of the Literature
by Giulia Pileggi, Sabrina Mariani, Valentina De Santis, Gianluca Maiorana, Federica Lubrano Lobianco, Chiara Togni, Monica Piedimonte, Caterina Tatarelli, Esmeralda Conte, Arianna di Napoli, Emanuela Pilozzi, Evelina Rogges, Agostino Tafuri and Giovanna Palumbo
Hemato 2025, 6(3), 18; https://doi.org/10.3390/hemato6030018 - 26 Jun 2025
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Abstract
Langerhans cell sarcoma (LCS) is a rare and aggressive neoplasm characterized by a clonal proliferation of Langerhans cells (LCs), with multi-organ involvement and poor prognosis. Diagnostic challenges arise from its rarity and overlapping features with Langerhans cell histiocytosis (LCH), requiring immunophenotypic and histological [...] Read more.
Langerhans cell sarcoma (LCS) is a rare and aggressive neoplasm characterized by a clonal proliferation of Langerhans cells (LCs), with multi-organ involvement and poor prognosis. Diagnostic challenges arise from its rarity and overlapping features with Langerhans cell histiocytosis (LCH), requiring immunophenotypic and histological analysis for differentiation. This case report discusses a 67-year-old male with multi-organ LCS involvement. Diagnosis was confirmed via liver biopsy and genetic analysis, revealing a MAP2K1 mutation. Treatment with subcutaneous cladribine and dexamethasone resulted in significant clinical and radiological improvement, despite hematological toxicity due to an underlying myelodysplastic neoplasm (MDS). This case proves the potential efficacy of cladribine for disseminated LCS and highlights the necessity for further research into optimal therapeutic approaches for this rare malignancy. Full article
(This article belongs to the Special Issue Hematopathology: Rare Hematological Diseases)
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