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How I treat hemophagocytic lymphohistiocytosis in the adult patient.成人噬血细胞性淋巴组织细胞增生症的治疗方法。
Blood. 2015 May 7;125(19):2908-14. doi: 10.1182/blood-2015-01-551622. Epub 2015 Mar 10.
2
Marked hyperferritinemia does not predict for HLH in the adult population.显著的高血铁蛋白血症不能预测成人 HLH。
Blood. 2015 Mar 5;125(10):1548-52. doi: 10.1182/blood-2014-10-602607. Epub 2015 Jan 8.
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Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients.成人反应性噬血细胞综合征:162 例回顾性分析。
Am J Med. 2014 Nov;127(11):1118-1125. doi: 10.1016/j.amjmed.2014.04.034. Epub 2014 May 14.
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Adult haemophagocytic syndrome.成人噬血细胞性淋巴组织细胞增生症。
Lancet. 2014 Apr 26;383(9927):1503-1516. doi: 10.1016/S0140-6736(13)61048-X. Epub 2013 Nov 27.
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Approach to hemophagocytic syndromes.噬血细胞综合征的处理方法。
Hematology Am Soc Hematol Educ Program. 2011;2011:178-83. doi: 10.1182/asheducation-2011.1.178.
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Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol.噬血细胞性淋巴组织细胞增生症的化免疫治疗:HLH-94 治疗方案的长期结果。
Blood. 2011 Oct 27;118(17):4577-84. doi: 10.1182/blood-2011-06-356261. Epub 2011 Sep 6.
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Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis.铁蛋白水平高度升高与噬血细胞性淋巴组织细胞增生症的诊断
Pediatr Blood Cancer. 2008 Jun;50(6):1227-35. doi: 10.1002/pbc.21423.
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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.HLH-2004:噬血细胞性淋巴组织细胞增生症的诊断与治疗指南。
Pediatr Blood Cancer. 2007 Feb;48(2):124-31. doi: 10.1002/pbc.21039.
9
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation.采用HLH - 94免疫化疗及骨髓移植治疗噬血细胞性淋巴组织细胞增生症。
Blood. 2002 Oct 1;100(7):2367-73. doi: 10.1182/blood-2002-01-0172.
10
Postanginal septicaemia (Lemmiere's disease) complicated by haemophagocytosis.咽后败血症(勒米埃尔氏病)合并噬血细胞现象。
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噬血细胞性淋巴组织细胞增生症的罕见病因:梭杆菌感染——病例报告及文献复习

A Rare Cause of Hemophagocytic Lymphohistiocytosis: Fusobacterium Infection-A Case Report and Review of the Literature.

作者信息

Mohyuddin Ghulam Rehman, Male Heather J

机构信息

Kansas University Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.

Division of Hematologic Malignancies and Cellular Therapeutics (HMCT), Department of Internal Medicine, University of Kansas Cancer Center, 2330 Shawnee Mission Parkway, Mailstop 5003, Westwood, KS 66205, USA.

出版信息

Case Rep Hematol. 2016;2016:4839146. doi: 10.1155/2016/4839146. Epub 2016 Aug 3.

DOI:10.1155/2016/4839146
PMID:27563473
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4987471/
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by excessive activation of the immune system. Bacterial infections are very rare precipitants of this disease. A 19-year-old gentleman presented with headache, fatigue, and malaise. He was found to be hypotensive, tachycardic, and febrile. Broad spectrum antibiotics were initiated, and a lumbar puncture ruled out meningitis. Patient progressively developed shock that required use of vasopressors, as well as renal and respiratory failure. Blood cultures grew Fusobacterium necrophorum. Given continued fevers despite appropriate antimicrobials, a bone marrow biopsy was performed revealing increased histiocytes with hemophagocytosis. Dexamethasone was added with dramatic clinical improvement. Our case highlights Fusobacterium as a rare precipitant of HLH and proves that a high index of clinical suspicion is crucial for early diagnosis of HLH, allowing for prompt initiation of HLH-specific immunosuppressive therapy that can be life-saving.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的综合征,其特征为免疫系统过度激活。细菌感染是该疾病非常罕见的诱因。一名19岁男性出现头痛、乏力和不适症状。检查发现他有低血压、心动过速和发热。开始使用广谱抗生素,腰椎穿刺排除了脑膜炎。患者逐渐发展为休克,需要使用血管升压药,同时出现了肾衰竭和呼吸衰竭。血培养结果显示坏死梭杆菌生长。尽管使用了适当的抗菌药物,患者仍持续发热,于是进行了骨髓活检,结果显示组织细胞增多并伴有噬血细胞现象。加用地塞米松后临床症状显著改善。我们的病例突出了坏死梭杆菌作为HLH罕见诱因的情况,并证明高度的临床怀疑对于HLH的早期诊断至关重要,从而能够及时启动可挽救生命的HLH特异性免疫抑制治疗。