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噬血细胞性淋巴组织细胞增生症:一种难以捉摸的综合征。

Haemophagocytic lymphohistiocytosis: an elusive syndrome.

作者信息

Thomas William, Veer Mars Van't, Besser Martin

机构信息

Addenbrooke's Hospital, Cambridge, UK

Addenbrooke's Hospital, Cambridge, UK.

出版信息

Clin Med (Lond). 2016 Oct;16(5):432-436. doi: 10.7861/clinmedicine.16-5-432.

DOI:10.7861/clinmedicine.16-5-432
PMID:27697804
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6297301/
Abstract

Haemophagocytic lymphohistiocytosis is a rare inflammatory condition. It can present in adult general medical patients and is a challenging diagnostic conundrum. This article provides an overview of the pathophysiology and clinical presentation of the syndrome for the general physician who will be rarely confronted with this problem but will have to act promptly when the situation arises. Treatment is also briefly discussed, although this usually occurs in a specialist setting after the diagnosis has been established.

摘要

噬血细胞性淋巴组织细胞增生症是一种罕见的炎症性疾病。它可出现在成年普通内科患者中,是一个具有挑战性的诊断难题。本文为普通内科医生概述了该综合征的病理生理学和临床表现,这类医生很少会遇到这个问题,但一旦出现这种情况必须迅速采取行动。文中也简要讨论了治疗方法,不过这通常是在确诊后在专科环境中进行。

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本文引用的文献

1
Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis.噬血细胞性淋巴组织细胞增生症成人的预后因素和转归。
Mayo Clin Proc. 2014 Apr;89(4):484-92. doi: 10.1016/j.mayocp.2013.12.012. Epub 2014 Feb 26.
2
Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens.利妥昔单抗联合化疗免疫治疗方案治疗 EBV 诱导的噬血细胞性淋巴组织细胞增生症。
Br J Haematol. 2013 Aug;162(3):376-82. doi: 10.1111/bjh.12386. Epub 2013 May 21.
3
Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis.疑似噬血细胞性淋巴组织细胞增生症患者的诊断评估。
Br J Haematol. 2013 Feb;160(3):275-87. doi: 10.1111/bjh.12138. Epub 2012 Dec 4.
4
Familial hemophagocytic lymphohistiocytosis may present during adulthood: clinical and genetic features of a small series.家族性噬血细胞性淋巴组织细胞增生症可发生于成年期:一系列小病例的临床和遗传学特征。
PLoS One. 2012;7(9):e44649. doi: 10.1371/journal.pone.0044649. Epub 2012 Sep 7.
5
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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Blood. 2011 Oct 13;118(15):4041-52. doi: 10.1182/blood-2011-03-278127. Epub 2011 Aug 9.
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Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation.降低强度的预处理方案可显著改善噬血细胞性淋巴组织细胞增生症患者接受异基因造血细胞移植后的生存情况。
Blood. 2010 Dec 23;116(26):5824-31. doi: 10.1182/blood-2010-04-282392. Epub 2010 Sep 20.
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Haematologica. 2010 Apr;95(4):538-41. doi: 10.3324/haematol.2009.019562.
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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.
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Hodgkin's lymphoma, Epstein-Barr virus reactivation and fatal haemophagocytic syndrome.霍奇金淋巴瘤、爱泼斯坦-巴尔病毒再激活与致命性噬血细胞综合征。
J Intern Med. 2004 Feb;255(2):289-95. doi: 10.1046/j.0954-6820.2003.01249.x.