Dommann-Scherrer C C, Kurer S B, Zimmermann D R, Odermatt B F, Dours-Zimmermann M T, Briner J, Heitz P U
Department of Pathology, University Hospital of Zurich, Switzerland.
Virchows Arch. 1995;426(6):629-34. doi: 10.1007/BF00192119.
We report on a patient with a rare hepatosplenic gamma delta T-cell lymphoma (gamma delta TCL) presenting clinically with B-symptoms, hepatosplenomegaly and pancytopenia. During the initial stage of the disease the sparse malignant cells could not be detected histologically. Furthermore, their identification was obscured by massive macrophage proliferation with haemophagocytosis in the spleen. Diagnosis was established by detection of a clonal T-cell receptor (TcR) rearrangement and, retrospectively, by demonstration of rare cells expressing and aberrant T-cell phenotype. The findings in this patient emphasize that minimal neoplastic T-cell infiltrates can lead to severe clinical symptoms. Initial biopsy findings may be misinterpreted as benign. Gamma delta TCL may elaborate lymphokines that suppress haematopoiesis, leading to pancytopenia and macrophage proliferation.
我们报告了1例罕见的肝脾γδ T细胞淋巴瘤（γδ TCL）患者，临床表现为B症状、肝脾肿大和全血细胞减少。在疾病初期，组织学检查未能检测到稀疏的恶性细胞。此外，脾脏中大量巨噬细胞增生伴噬血细胞现象掩盖了它们的识别。通过检测克隆性T细胞受体（TcR）重排确诊，回顾性分析通过证实罕见细胞表达异常T细胞表型确诊。该患者的发现强调，微小的肿瘤性T细胞浸润可导致严重的临床症状。最初的活检结果可能被误诊为良性。γδ TCL可能分泌抑制造血的淋巴因子，导致全血细胞减少和巨噬细胞增生。
