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Articles Published Processes
10/20/2014 10:04:00 AM | Browse: 1489 | Download: 1393
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Received |
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2014-03-09 17:10 |
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Peer-Review Started |
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2014-03-10 08:45 |
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To Make the First Decision |
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2014-04-28 14:34 |
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Return for Revision |
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2014-04-30 15:00 |
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Revised |
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2014-05-09 16:37 |
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Second Decision |
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2014-06-18 14:58 |
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Accepted by Journal Editor-in-Chief |
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Accepted by Executive Editor-in-Chief |
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2014-06-18 15:17 |
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Articles in Press |
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2014-06-18 15:42 |
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Publication Fee Transferred |
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Edit the Manuscript by Language Editor |
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2014-06-24 03:01 |
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Typeset the Manuscript |
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2014-09-28 17:48 |
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Publish the Manuscript Online |
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2014-10-20 10:04 |
Category |
Hematology |
Manuscript Type |
Review |
Article Title |
Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment
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Manuscript Source |
Invited Manuscript |
All Author List |
Shinsaku Imashuku |
Funding Agency and Grant Number |
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Corresponding Author |
Shinsaku Imashuku, MD, PhD
Consultant to the Department of Laboratory Medicine, Uji-Tokushukai Medical Center
86 Kasugamori, Ogura-cho, Uji, Kyoto, Japan 611-0042
Tel: +81-774-20-1111; FAX +81-0774-20-2336
E-mail: shinim95@mbox.kyoto-inet.or.jp
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Key Words |
hemophagocytic lymphohistiocytosis; ; molecular diagnosis; hematopoietic stem-cell transplantation; cyclosporine A; rituximab |
Core Tip |
This review discusses the diagnostic criteria for HLH, the algorithms used to identify the underlying immune defects at the molecular level, and the optimal therapeutic approaches. For any HLH cases, a screening for primary HLH should be made following the diagnostic algorithm. During the process, immunosuppressive therapy should be started to control the hypercytokinemia with steroids, CSA, or IVIG, and if primary HLH is confirmed, immunochemotherapy with a regimen containing etoposide or ATG should be given. Supportive measures to control hemorrhage/organ dysfunction are also required. In cases of primary HLH or secondary/refractory HLH, timely allogeneic hematopoietic stem-cell transplantation is recommended. |
Publish Date |
2014-10-20 10:04 |
Citation |
Imashuku S. Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment. World J Hematol 2014; 3(3): 71-84 |
URL |
http://www.wjgnet.com/2218-6204/full/v3/i3/71.htm |
DOI |
http://dx.doi.org/10.5315/wjh.v3.i3.71 |
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