本文分析经尸检证实为本病的14例患儿的病理与临床特征。其表现:(1)肝脾重量多数超过同龄儿;(2)深部淋巴结肿大受侵率最高,而皮肤受累的阳性率又较浅表淋巴结为高,因此建议常规作皮肤活检;(3)局部皮下出现特大肿块,往往系瘤细胞高度集中浸润所致。本文还通过1例仅1日龄的新生儿初步探讨与遗传的关系。 This article analyzes the autopsy confirmed the disease-based 14 cases of children with pathological and clinical features. Its performance: (1) the majority of liver and spleen weight more than their peers; (2) the most affected deep lymph node enlargement, while the positive rate of skin involvement and superficial lymph nodes is high, so the proposed routine skin biopsy; (3) Local subcutaneous large hyperplasia, often Department of tumor cells highly concentrated infiltration caused. This article also through a case of only 1 day old newborn prenatal genetic relationship.