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本文报告我院在1972年7月、1977年9月收治的2例家族性噬血性组织细胞增生症病婴,复习收集到的国外文献报道32例(尚未收集到国内报告),并加以讨论。本病大多数起病于婴儿,是恶性组织细胞增生症的一种罕见类型,常呈家族性发病。主要临床特征是:显著的苍白,不规则发热,肝脾肿大和进行性全血细胞减少,并常伴有中枢神经系统症状。病理特征为全身多脏器广泛的噬血性(主要吞噬红细胞)的组织细胞浸润。病因不明,生前诊断困难,目前尚无特效治疗。须与其它恶性组织细胞病鉴别。本文着重讨论其生前诊断的要点。
This article reports in our hospital in July 1972, September 1977 admitted to 2 cases of hemophagocytic hematopoietic disease infants, review collected in foreign literature reported 32 cases (not yet collected domestic reports), and to be discussed. Most of the disease onset in infants, is a rare type of malignant histiocytosis, often familial disease. The main clinical features are: significant pale, irregular fever, hepatosplenomegaly and progressive pancytopenia, and often accompanied by central nervous system symptoms. Pathology is characterized by extensive hemophagocytic (predominantly phagocytosed erythrocytes) tissue-cell infiltration of the whole body and multiple organs. Etiology is unknown, prenatal diagnosis is difficult, there is no effective treatment. Must be differentiated from other malignant cytopathies. This article focuses on the main points of its lifetime diagnosis.