Kobayashi Y, Uehara S, Inamori K, Shirato R, Ozawa K, Sklar J, Asano S
Department of Hematology and Oncology, University of Tokyo, Japan.
Int J Hematol. 1996 Aug;64(2):135-42. doi: 10.1016/0925-5710(96)00477-x.
Hemophagocytic syndrome is a proliferative disorder of an activated monocyte-macrophage system and is characterized by fever, hepato-splenomegaly and pancytopenia. The serum level of interferon-gamma in the syndrome is increased but its origin is unknown. Here we describe a case of NK cell leukemia with hemophagocytic syndrome with elevated serum level of interferon-gamma. The levels of various cytokines were monitored during the course and statistic analysis was performed. To identify the clonal component, the NK cell fraction was sorted from the mononuclear layer and was subjected to Southern blot hybridization with a probe for EB virus tandem repeats. The fraction was also stimulated with interleukin-2 and the level of interferon-gamma in the conditioned medium was measured. Levels of M-CSF and interferon-gamma were significantly correlated with the degree of clinical manifestations and laboratory data. Southern blot hybridization revealed monoclonality of an NK cell fraction. The fraction also released interferon-gamma. Since macrophage can be activated through cytokines, the hemophagocytosis might have been triggered by factor(s) released from the abnormal NK cell clone at least in this case.
噬血细胞综合征是一种活化的单核细胞-巨噬细胞系统的增殖性疾病，其特征为发热、肝脾肿大和全血细胞减少。该综合征中干扰素-γ的血清水平升高，但其来源不明。在此，我们描述一例伴有噬血细胞综合征且血清干扰素-γ水平升高的自然杀伤（NK）细胞白血病病例。在病程中监测了各种细胞因子的水平，并进行了统计学分析。为鉴定克隆成分，从单核细胞层中分离出NK细胞部分，并用针对EB病毒串联重复序列的探针进行Southern印迹杂交。该部分细胞还用白细胞介素-2刺激，并检测条件培养基中干扰素-γ的水平。巨噬细胞集落刺激因子（M-CSF）和干扰素-γ的水平与临床表现和实验室数据的程度显著相关。Southern印迹杂交显示NK细胞部分具有单克隆性。该部分细胞也释放干扰素-γ。由于巨噬细胞可通过细胞因子被激活，至少在本病例中，噬血细胞现象可能是由异常NK细胞克隆释放的因子触发的。
