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Advances in the diagnosis and management of haemophagocytic lymphohistiocytosis: a review of literature

Publication: LymphoSign Journal
2 February 2018

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, triggered by the excessive stimulation of lymphocytes and macrophages producing abnormally increased levels of cytokines. The diagnosis can be challenging due to overlapping signs and symptoms with other diseases. Therefore, early detection and prompt initiation of treatment is crucial for better survival. There are mainly 2 forms of HLH, primary (genetic) and secondary (acquired). Recent advances in the diagnosis and treatment modalities have led to better understanding of HLH. Here, we present a concise review of the literature with recent updates in pathogenesis, diagnostic modalities, and treatment of HLH.
Statement of novelty: In this concise review of HLH, we discuss from the historical background to contemporary diagnostic modalities, as well as recent updates on treatment.

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cover image LymphoSign Journal
LymphoSign Journal
Volume 5Number 1March 2018
Pages: 1 - 15

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Received: 4 September 2017
Accepted: 31 January 2018
Accepted manuscript online: 2 February 2018

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Saima Alvi
Pediatric Oncology, Allan Blair Cancer Centre, Regina, SK
Ahmed Naqvi [email protected]
Haematology/Oncology, The Hospital for Sick Children, Toronto, ON

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