Hu Zhihong, Li Shaoying, Medeiros L Jeffrey, Sun Tsieh
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030.
Hum Pathol. 2017 Jul;65:175-179. doi: 10.1016/j.humpath.2016.12.030. Epub 2017 Feb 21.
T-prolymphocytic leukemia (T-PLL) is a rare mature T-cell neoplasm characterized by proliferation of prolymphocytes. Most cases involve the T-cell leukemia-1 (TCL1) gene at 14q11.2 resulting in overexpression of TCL-1, which is helpful for distinguishing T-PLL from other T-cell neoplasms. We report a patient with T-PLL whose leukemic cells were positive for TCL-1 by immunohistochemistry but with a normal karyotype. The patient had anti-CD52 antibody therapy for 12 weeks. In a follow-up bone marrow biopsy specimen, numerous TCL-1-positive cells were present, which raised the differential diagnosis of residual T-PLL. However, further immunophenotypic studies confirmed that these cells were hematogones. Therefore a diagnosis of recovering bone marrow was established. The patient underwent stem cell transplant and is now in complete remission. This case demonstrates that hematogones can express TCL-1, and this knowledge is very important for the differential diagnosis in the follow-up marrow of T-PLL patients.
T 原淋巴细胞白血病（T-PLL）是一种罕见的成熟 T 细胞肿瘤，其特征为原淋巴细胞增殖。大多数病例涉及位于 14q11.2 的 T 细胞白血病-1（TCL1）基因，导致 TCL-1 过度表达，这有助于将 T-PLL 与其他 T 细胞肿瘤区分开来。我们报告了 1 例 T-PLL 患者，其白血病细胞经免疫组织化学检测 TCL-1 呈阳性，但核型正常。该患者接受了抗 CD52 抗体治疗 12 周。在后续的骨髓活检标本中，存在大量 TCL-1 阳性细胞，这引发了对残留 T-PLL 的鉴别诊断。然而，进一步的免疫表型研究证实这些细胞是造血祖细胞。因此确诊为骨髓恢复。该患者接受了干细胞移植，目前处于完全缓解状态。此病例表明造血祖细胞可表达 TCL-1，这一认识对于 T-PLL 患者后续骨髓的鉴别诊断非常重要。
