Aslan Y, Erduran E, Gedik Y, Mocan H, Yildiran A
Department of Pediatrics, Karadeniz (Black Sea) Technical University, Faculty of Medicine, Trabzon, Turkey.
Acta Haematol. 1996;96(2):105-7. doi: 10.1159/000203725.
The Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disorder. Some cases with CHS develop the accelerated phase characterized by pancytopenia, high fever and lymphohistiocytic infiltration of liver, spleen and lymph nodes. The treatment of the accelerated phase of CHS is difficult. We describe a case with CHS in the accelerated phase who had multiple polyposis and pulmonary infiltration that was probably due to involvement of CHS. She was successfully treated with high-dose methylprednisolone at her first admission. At her second admission, splenectomy was performed to remove hypersplenism, and her clinical, radiological and hematological findings improved significantly.
切-东综合征（CHS）是一种罕见的常染色体隐性免疫缺陷病。部分CHS病例会发展为加速期，其特征为全血细胞减少、高热以及肝脏、脾脏和淋巴结的淋巴细胞组织细胞浸润。CHS加速期的治疗颇具难度。我们报告一例处于加速期的CHS病例，该患者伴有多发性息肉病和肺部浸润，这可能是由于CHS累及所致。她首次入院时接受大剂量甲泼尼龙治疗取得成功。第二次入院时，进行了脾切除术以消除脾功能亢进，其临床、影像学和血液学检查结果均有显著改善。
