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Review Article
- T-lymphoblastic leukemia/lymphoma: a comprehensive review of pathology, molecular features, and differential diagnosis
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David Danielson, Ian T. Lagerstrom, Max Rogers, Kyle Simon, Nadine S. Aguilera, Aaron Auerbach
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J Pathol Transl Med. 2026;60(5):502-515. Published online September 15, 2026
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DOI: https://doi.org/10.4132/jptm.2026.08.01
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Abstract
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- T-lymphoblastic leukemia/lymphoma (T-ALL/LBL) is an aggressive neoplasm of immature T-lymphoid precursors that is classified as T-cell acute lymphoblastic leukemia when the primary site of involvement is the bone marrow and peripheral blood and as T-cell lymphoblastic lymphoma when it presents as a solid mass, most commonly in the anterior mediastinum. The neoplastic cells are defined by expression of T-lineage antigens (cytoplasmic or surface CD3) together with one or more markers of immaturity (terminal deoxynucleotidyl transferase, CD1a, CD34, CD99, or CD117) and must not fulfill criteria for early T-cell precursor acute lymphoblastic leukemia (ALL). This entity shows a marked male predominance (male:female ≈ 2:1) and predominantly affects children, adolescents, and young adults, accounting for approximately 15% of childhood ALL and 20%–25% of adult ALL cases. Pathologic diagnosis increasingly relies on recurrent molecular alterations such as activating NOTCH1 mutations (>60% of cases), transcription factor rearrangements, and cell-cycle regulator inactivation. Although intensive multiagent chemotherapy regimens have substantially improved outcomes, particularly in pediatric patients, adults and high-risk molecular subgroups continue to experience inferior survival. This review provides a practical, pathology-oriented update on the epidemiology, pathogenesis, diagnostic criteria, differential diagnosis, prognostic factors, and current treatment approaches for T-ALL/LBL.
Case Report
- A Case of Malignant Lymphoma Misdiagnosed as Focal Lymphoid Hyperplasia in the Esophagus.
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Sook Keum Chung, Young Hyeh Ko, Chan Keum Park, Jung Dal Lee
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Korean J Pathol. 1995;29(3):393-398.
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Abstract
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- Esophageal involvement by malignant lymphoma is extremely rare. A case of follicular lymphoma of the esophagus, misdiagnosed as focal lymphoid hyperplasia (pseudolymphoma) in a 72-year-old man is presented. The esophagogram revealed diffuse narrowing of the lumen in the middle and distal portion without ulceration. The resected esophagus showed mural thickening without any remarkable mucosal change.
Microscopically, the esophagus showed scattered follicular lymphoid aggregates in the submucosa, extending into periadventitial fat tissue. Most follicles were devoid of germinal center and consisted of loosely aggregated small cleaved cells without atypia. The surrounding stroma of the submucosa showed dense fibrosis entrapping the infiltrating small lymphocytes in a "indian-file" appearance. There were some reactive follicles with germinal center. In the lamina propria, many plasma cells and a few eosinophils were infiltrated. The gene rearrangement study showed rearranged band for Jk probe which confirmed monoclonal B-cell nature of infiltrated small lymphoid cells. The small cleaved lymphocytes arranged in follicles were positive for L26 and bcl-2 protein. This case demonstrated the necessity of immunophenotypic and gene rearrangement study in the diagnosis of pseudolymphoma in the digestive tract.
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