Rubin C M, Burke B A, McKenna R W, McClain K L, White J G, Nesbit M E, Filipovich A H
Cancer. 1985 Aug 1;56(3):524-30. doi: 10.1002/1097-0142(19850801)56:3<524::aid-cncr2820560320>3.0.co;2-z.
The clinical and pathologic findings of four patients with Chediak-Higashi syndrome in the accelerated phase were studied in order to clarify the nature of this enigmatic process. Fever, lymphadenopathy, hepatosplenomegaly, and cytopenias were present in every patient. All cases demonstrated extensive parenchymal infiltrates in many organs composed of benign-appearing histiocytes manifesting hemophagocytosis accompanied by lymphocytes and plasma cells. Studies in one patient suggested a viral etiology with the findings of a low blood lymphocyte OKT4 to OKT8 ratio, acquired loss of lymphocyte response to mitogens, the presence of Epstein-Barr virus genome in the mononuclear cells of lymph node, blood, and bone marrow, and possible clinical responses to acyclovir. It is concluded that the accelerated phase of Chediak-Higashi syndrome may be the clinicopathologic expression of the virus-associated hemophagocytic syndrome.
为阐明切迪阿克-东综合征加速期这一神秘过程的本质，对4例处于加速期的切迪阿克-东综合征患者的临床和病理表现进行了研究。每位患者均出现发热、淋巴结病、肝脾肿大和血细胞减少。所有病例均显示许多器官存在广泛的实质浸润，浸润细胞为外观良性的组织细胞，表现为噬血细胞现象，并伴有淋巴细胞和浆细胞。对1例患者的研究提示存在病毒病因，表现为血液淋巴细胞OKT4与OKT8比值降低、淋巴细胞对有丝分裂原的反应性获得性丧失、淋巴结、血液和骨髓单核细胞中存在爱泼斯坦-巴尔病毒基因组，以及对阿昔洛韦可能出现临床反应。结论是，切迪阿克-东综合征加速期可能是病毒相关噬血细胞综合征的临床病理表现。
