Valenzuela R, Aikawa M, O'Regan S, Makker S
Am J Clin Pathol. 1976 Apr;65(4):483-94. doi: 10.1093/ajcp/65.4.483.
Clinicopathologic observations in the case of a black infant with Chediak-Higashi syndrome are reported. Light and electron microscopic examination of spleen, liver and lymph nodes revealed abnormal large lysosomes as well as marked erythrophagocytosis without hemosiderosis in infiltrating histiocytes and Kupffer cells. In addition, there were abnormal ocular findings. It is suggested that the erythrophagocytosis without hemosiderosis might also be a specific anatomic expression of this disease due to defective lysosomal digestion of phagocytosed erythrocytes.
报告了一名患有切-希二氏综合征的黑人婴儿的临床病理观察结果。对脾脏、肝脏和淋巴结进行光镜和电镜检查发现，浸润的组织细胞和库普弗细胞中有异常大的溶酶体以及明显的红细胞吞噬现象，但无含铁血黄素沉着。此外，还有异常的眼部表现。提示无含铁血黄素沉着的红细胞吞噬现象也可能是由于吞噬的红细胞溶酶体消化缺陷导致的该疾病的一种特殊解剖学表现。
