Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases
Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases
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DOI：
10.1111/j.1600-0609.1997.tb00984.x
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发表时间：
1997-10
影响因子：
3.1
通讯作者：
K. Kaito;M. Kobayashi;T. Katayama;H. Otsubo;Y. Ogasawara;T. Sekita;A. Saeki;M. Sakamoto;K. Nishiwaki;H. Masuoka;T. Shimada;M. Yoshida;T. Hosoya
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文献类型：
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作者：
K. Kaito;M. Kobayashi;T. Katayama;H. Otsubo;Y. Ogasawara;T. Sekita;A. Saeki;M. Sakamoto;K. Nishiwaki;H. Masuoka;T. Shimada;M. Yoshida;T. Hosoya
摘要：噬血细胞综合征（HPS）主要表现为发热、全血细胞减少、肝功能损害及各器官噬血细胞增多。虽然成人HPS有两种主要分类，恶性和反应性组织细胞增生症，但通常很难区分这些疾病。我们分析了HPS患者的实验室数据，以评估预后因素。34例患者中，14例存活，20例死亡。存活者的中位年龄为29.6 ± 11.5岁，明显小于死亡者（54.7 ± 17.8岁）。20例患者没有明显的基础疾病，其他13例有血液恶性肿瘤或病毒感染。实验室数据的比较显示，非幸存者有显着较低的血红蛋白和血小板值入院。治疗期间，贫血和血小板减少症恶化、转氨酶和胆汁酶升高同样更为显著。与死亡相关的风险因素为：年龄超过30岁、存在弥散性血管内凝血、铁蛋白和β2微球蛋白升高、贫血伴血小板减少症和黄疸。我们的数据表明，HPS患者和任何这些危险因素应积极治疗充分的化疗和支持性治疗。
Abstract: Hemophagocytic syndrome (HPS) presents with fever, pancytopenia, liver dysfunction and increase in hemophagocytic histiocytes in various organs. Although there are two major classifications of HPS in adults, malignant and reactive histiocytosis, it is often very difficult to distinguish between these disorders. We analyzed the laboratory data of patients with HPS to evaluate prognostic factors. Of 34 patients, 14 survived, and 20 died. The median age of survivors was 29.6 ± 11.5 yr significantly younger than those who died (54.7 ± 17.8 yr). Twenty patients had no obvious underlying disease, the other 13 had hematological malignancies or viral infections. Comparison of laboratory data revealed that nonsurvivors had significantly lower Hb and platelet values on admission. During treatment, worsening of anemia and thrombocytopenia, increase of transaminase and biliary enzymes were similarly more prominent. Risk factors associated with death were: age over 30 yr, presence of disseminated intravascular coagulation, increased ferritin and β2‐microglobulin, anemia accompanied by thrombocytopenia and jaundice. Our data suggests that patients with HPS and any of these risk factors should be treated aggressively with sufficient chemotherapy and supportive care.