Autoimmune-associated hemophagocytic syndrome
Autoimmune-associated hemophagocytic syndrome
复制标题
自身免疫相关噬血细胞综合征
DOI：
10.3109/s10165-004-0293-6
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发表时间：
2004
影响因子：
2.2
通讯作者：
Shotai Kobayashi
中科院分区：
文献类型：
--
作者：
S. Kumakura;H. Ishikura;M. Kondo;Y. Murakawa;J. Masuda;Shotai Kobayashi
噬血细胞综合征（Hemophagocytic syndrome， HPS）是一种临床病理症状，其特征是骨髓和其他网状内皮系统中组织细胞的活化，并伴有明显的噬血细胞症。HPS的发生通常与感染和淋巴瘤等潜在疾病有关。最近，我们描述了患有自身免疫性疾病的HPS患者。在这些病例中，没有潜在感染和恶性肿瘤的证据，HPS的发生与活动性自身免疫性疾病有关。基于这些观察，我们描述了自身免疫相关的噬血细胞综合征（AAHS）。这种疾病实体正变得越来越为人所知，呈现与临床AAHS相一致特征的病例报告也在增加。在此，我们根据我们的资料和文献综述AAHS的临床方面、机制、诊断和治疗。
Hemophagocytic syndrome (HPS) is a clinicopathological condition characterized by the activation of histiocytes with prominent hemophagocytosis in bone marrow and other reticuloendothelial systems. The occurrence of HPS is usually associated with underlying disorders such as infection and lymphoma. Recently, we described patients with autoimmune disease who developed HPS. In these cases there was no evidence of underlying infection and malignancy, and the occurrences of HPS were associated with active autoimmune disease. Based on these observations, we described autoimmune-associated hemophagocytic syndrome (AAHS). This disease entity is becoming better known, and case reports presenting features compatible with clinical AAHS are increasing. Here, we review the clinical aspects, mechanisms, diagnosis, and treatment of AAHS according to our data and that in the literature.
影响因子：
4.4
作者：
V. Fadok;J. Savill;C. Haslett;D. Bratton;D. Doherty;Priscilla A. Campbell;Peter M. Henson
通讯作者：
V. Fadok;J. Savill;C. Haslett;D. Bratton;D. Doherty;Priscilla A. Campbell;Peter M. Henson