噬血细胞综合征研究进展

Hemophagocytic Syndrome: Recent Advances

噬血细胞综合征 ( HPS)是 T细胞介导的组织细胞异常增生 ,部分病例已证实 TCRγ或 β基因的单克隆重排。半数病例进展急剧 ,预后不良 ,缺乏特效治疗 ;重症病例早期异基因骨髓移植优于常规化疗或免疫抑制治疗。临床和细胞形态学、组织学与恶性组织细胞增生症 ( MH )十分相似 ,尚缺乏特异鉴别标记。 HPS比 MH多见 ,部分病例常误认为 MH。本文就 HPS有关资料 ,对该病的发病学、临床表现、实验室检查。

Hemophagocytic syndrome( HPS) is an abnormal systemic proliferation of histocyte lineage mediated by Tc cells. It is confirmed that T cell receptor ( TCR) γ or β gene monoclonal rearrangements are present in some patients. It has a rapidly deteriorating course in approximated half of the patients, the outcome is usually poor, response to treatment is unsatisfactory, early bone marrow ablative chemotherapy with allograft would be a better option than conventional chemotherapy or immunosuppression. Differential diagnosis with malignant histocytosis ( MH) is usually very difficult at clinical ,cellular morphological and pathohistological aspects. Specific differential markers are yet absence. Incidence of HPS is far exceed MH, Some patients of HPS are misdiagnosed as MH. The pathogenesis, clinical features, laboratory findings, treatment and outcome of HPS are reviewed in this article.