Fadilah S A, Raymond A A, Cheong S K, Amir M A
Division of Clinical Haematology and Stem Cell Transplantation, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.
Med J Malaysia. 2001 Dec;56(4):503-7.
A fulminant clinical presentation with high fever and hepatosplenomegaly, together with a course of worsening pancytopenia, coagulopathy and liver failure, is suggestive of the haem syndrome (HPS). Bone marrow examination is diagnostic. We present 3 cases of HPS associated with different aetiologies including acute Ebstein Barr virus infection, T cell lymphoma, and malignant histiocytosis. In all the cases, the diagnosis was made late and the patients succumbed before definitive therapy could be administered.
伴有高热和肝脾肿大的暴发性临床表现，以及全血细胞减少、凝血病和肝功能衰竭不断恶化的病程，提示为噬血细胞综合征（HPS）。骨髓检查具有诊断意义。我们报告3例与不同病因相关的HPS病例，包括急性EB病毒感染、T细胞淋巴瘤和恶性组织细胞增多症。在所有病例中，诊断均较晚，患者在能够进行确定性治疗之前死亡。
