Hemophagocytic syndrome in elderly patients with underlying autoimmune diseases
Hemophagocytic syndrome in elderly patients with underlying autoimmune diseases
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DOI：
10.1007/s10067-009-1086-2
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发表时间：
2009-04-01
影响因子：
3.4
通讯作者：
Nagai, Tomoko
中科院分区：
文献类型：
--
作者：
Tabata, Rie;Tabata, Chiharu;Nagai, Tomoko
自身免疫性疾病相关噬血细胞综合征（AAHS）患者的临床特征可能不同于其他病因的噬血细胞综合征（HPS），并提出了新的AAHS诊断标准。由于骨髓（BM）的情况是根据年龄的变化，在这里，我们回顾性分析了我们的病例与AAHS的老年患者，包括两个系统性红斑狼疮（SLE），三个埃文斯综合征，类风湿性关节炎（RA），桥本甲状腺炎，和自身免疫性胰腺炎。虽然只有2例SLE患者按经典标准诊断为HPS，但除1例RA外，其余患者均符合AAHS标准。除1例SLE患者外，7例患者对治疗反应良好，表现出血细胞自身抗体阳性，血清铁蛋白水平降低，BM成红细胞岛增加。我们认为，老年自身免疫性疾病患者骨髓涂片中出现巨噬细胞吞噬血细胞而无高铁蛋白血症时，应慎重诊断。
In patients with autoimmune disease-associated hemophagocytic syndrome (AAHS), the clinical features may differ from hemophagocytic syndrome (HPS) of other etiologies, and new criteria for AAHS have been proposed. Since bone marrow (BM) circumstances are changed according to aging, here we reviewed retrospectively our cases with AAHS in elderly patients, including two systemic lupus erythematosus (SLE), three Evans syndrome, one rheumatoid arthritis (RA), one Hashimoto thyroiditis, and one autoimmune pancreatitis. Although only two SLE patients were diagnosed as HPS by the classical criteria, the remaining patients except one RA met the criteria for AAHS. Seven patients except one SLE patient showed good response to therapy and demonstrated positive autoantibodies to blood cells, lower serum ferritin levels, and increased erythroblastic islands in the BM. We consider the diagnosis of AAHS should be carefully made when macrophages phagocytosing blood cells are observed in BM smear without hyperferritinemia in elderly patients with autoimmune diseases.