Mastovich S, Ratech H, Ware R E, Moore J O, Borowitz M J
Department of Pathology, Duke University Medical Center, Durham, NC.
Hum Pathol. 1994 Jan;25(1):102-8. doi: 10.1016/0046-8177(94)90179-1.
We describe a case of a middle-aged women who presented with anemia and mild hepatosplenomegaly and who was found to have an unusual peripheral T-cell lymphoma with only subtle morphologically abnormal but mature-appearing cells noted in the blood and bone marrow. Less than 2 years after diagnosis the patient presented with an increasing white blood cell count to 26 x 10(9)/L, and numerous blasts were noted in the periphery. Flow cytometry studies showed cells with an unusual T-cell phenotype expressing the gamma delta T-cell receptor and restricted expression of the V delta 1 but not the V delta 2 protein, indicating the clonal nature of the proliferation. A clonal T-cell receptor gene rearrangement was seen with a V delta 1 probe. The patient died and was found at autopsy to have extensive hepatic sinusoidal infiltration by abnormal cells. The histopathologic, immunophenotypic, and molecular findings are those of "hepatosplenic T-cell lymphoma." In spite of the striking morphologic change during the course of the patient's disease the same phenotype and clonal rearrangement were found both at initial diagnosis and during terminal phase, indicating that this change represented a blast-like transformation of the patient's original lymphoproliferative disorder.
我们描述了一例中年女性患者，该患者表现为贫血和轻度肝脾肿大，经检查发现患有一种不寻常的外周T细胞淋巴瘤，其血液和骨髓中仅见形态学上有细微异常但外观成熟的细胞。诊断后不到2年，患者白细胞计数升至26×10⁹/L，外周血中可见大量原始细胞。流式细胞术研究显示细胞具有不寻常的T细胞表型，表达γδ T细胞受体，Vδ1蛋白呈限制性表达而Vδ2蛋白不表达，提示增殖具有克隆性。用Vδ1探针检测到克隆性T细胞受体基因重排。患者死亡，尸检发现肝脏有广泛的异常细胞窦状浸润。组织病理学、免疫表型和分子学检查结果均符合“肝脾T细胞淋巴瘤”。尽管患者病程中形态学发生了显著变化，但初始诊断时和终末期均发现相同的表型和克隆性重排，表明这种变化代表了患者原始淋巴增殖性疾病的母细胞样转化。
