肠型恶性组织细胞增生症(附53例临床病理分析)

The Clinico-pathological Analysis of 53 Cases of Malignant Histiocytosis of the Intestine Type

本文对53例肠型恶性组织细胞增生症进行分析。包括国内文献报告42例及本组11例。发现肠恶组的临床病理特征为1.多发生在30岁以前,男∶女=2.6∶1;2.病程短,进展快,6个月内死亡者占60.7％;3.最常见症状为发热,腹痛,消化道出血与肠穿孔以及腹块等;4.病理形态表现为肠道病变多发及分布不规则,可胃肠道同时受累。局部有坏死,溃疡及穿孔,组织学观察可见病变处有各型恶性组织细胞浸润,彼此分离,胞浆内吞噬红细胞或细胞碎片的特征;5.造血器官受累较少见。对本病的诊断与鉴别诊断作了简要分析。

Fifty three cases of malignant histiocytosis of the intestine (MHI) were analysed, including 42 cases collected from the domestic literature and 11 added by the author. The clinico-pathologic features of MHI are as follows: (1) High incidence in patients less than 30 years of age and the ratio of male to female being 2.6:1 in this series; (2) Rapid progress with inevitable fatality, 60.7% of the cases died within 6 months of onset; (3)Fever, abdominal pain, alimentary tract hemorrhage, acute intestinal perforation and abdominal mass were the common clinical symptoms; (4) Morphologically, multiple involvements of the intestine with irregular distribution, local necrosis, ulceration and perforation were common. Histological examination generally rerveals prominant infiltration of the lesion by malignant histiocytes which usually characteristically show non-aggregation, non-coherency, and erythrophagocytosis; (5) Involvement of hematopoietic organs is relatively rare.