Swart Violet O, Zoghi Behyar
Department of Bone Marrow Transplant, Methodist Hospital, San Antonio, Texas, USA.
Case Rep Hematol. 2025 Jul 3;2025:7634316. doi: 10.1155/crh/7634316. eCollection 2025.
Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) is a high-risk and biologically distinct subset of T-cell acute lymphoblastic leukemia, typically characterized by leukemic involvement of bone marrow, peripheral blood, and mediastinal structures. Central nervous system (CNS) involvement is typically a secondary manifestation [1, 2, 3]. We report a rare case of isolated CNS presentation of ETP-ALL/LBL in a 73-year-old woman who presented with progressive neurological symptoms. Imaging revealed a large right frontal extra-axial mass. Systemic evaluation, including peripheral blood flow cytometry and CT imaging of chest, abdomen, and pelvis, showed no evidence ofsystemic disease. Surgical resection and subsequent histopathology confirmed the diagnosis of ETP-ALL/LBL. A bone marrow biopsy was deffered to the patient's preference and rapid clinical deterioration. This case underscores the diagnostic and therapeutic challenges of CNS-restricted leukemic presentations and highlights the need for early recognition and CNS-directed diagnostic evaluation.
早期T细胞前体急性淋巴细胞白血病/淋巴瘤（ETP-ALL/LBL）是T细胞急性淋巴细胞白血病的一个高危且生物学特性独特的亚群，其典型特征是白血病累及骨髓、外周血和纵隔结构。中枢神经系统（CNS）受累通常是一种继发表现[1,2,3]。我们报告了一例罕见的ETP-ALL/LBL孤立性CNS表现病例，患者为一名73岁女性，表现为进行性神经症状。影像学检查发现右侧额叶有一个大的轴外肿块。包括外周血流式细胞术以及胸部、腹部和骨盆CT成像在内的全身评估未发现系统性疾病证据。手术切除及后续组织病理学检查确诊为ETP-ALL/LBL。鉴于患者的意愿和快速的临床恶化情况，未进行骨髓活检。该病例强调了CNS局限性白血病表现的诊断和治疗挑战，并突出了早期识别和针对CNS的诊断评估的必要性。
