噬血细胞综合征的临床病理学观察

目的:研究噬血细胞综合征(HPS)的临床病理学特征.提高对本病诊断的认识。方法:回顾性分析35例HPS 的病史资料、实验室检查,同时结合骨髓涂片和骨髓塑包活检切片。结果:家族性噬血细胞性淋巴组织细胞增生症(FHL)4例(11.4%),均有家族史。感染相关性 HPS(IAHS)14例(40%),其中6例(17.1%)为病毒感染,另外8例(22.9%)为细菌感染。肿瘤相关性 HPS(MAHS)17例(48.6%),有3例(8.6%)出现神经症状,5例(14.3%)骨髓中检出肿瘤细胞,7例(20%)骨髓活检伴有骨髓纤维增生。结论:结合临床和骨髓病理学特征诊断 HPS.特别是 IAHS,有助于鉴别恶性组织细胞病.从而对判断患者预后有重要意义。

Clinicopathologic characteristics of hemophagocytic syndrome

Objective To research the clinicopathologic characteristics of hemophagocytic syndrome,and to im- prove our acquaintance in the diagnosis.Methods The data and the marrow smears in 35 cases of hemophagocytie syndrome were assayed retrospectively.Results Four cases (11.4%),all of them had a family history,were diagnosed as familia hemophagocytic lymphohistiocytosis.Fourteen cases (40%) were diagnosed as infection-associated hemophagocytic syndrome,and 6 (17.1%) of them were viral infectious and the other 8(22.9%) were bacterial infectious.The other 17 cases(48.6%) were diagnosed as malignancy-associated hemophagoeytic syndrome,and 3 (8.6%) of them had neurosis. Tumor cells in the marrow smears were found in 5 cases (14.3%) anti bone marrow fibrosis in 7 cases (20%).Conclu- sions It is helpful to differentiate the malignant histiocytosis from the hemophagocytic syndrome,especially the infection- associated hemophagocytic syndrome,which is diagnosed by combining the clinical data with the pathological characteris- tics.Therefore,it is important to predict the prognosis.