Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options

Ellen Brisse; Patrick Matthys; Carine H. Wouters

doi:10.1111/bjh.14144

journal article Jun 12, 2016

Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options

Ellen Brisse Patrick Matthys

Carine H. Wouters

British Journal of Haematology Vol. 174 No. 2 pp. 175-187 · Wiley

View at Publisher Save 10.1111/bjh.14144

Abstract

SummaryThe cytokine storm syndrome ‘haemophagocytic lymphohistiocytosis’ (HLH) is an under‐recognized hyperinflammatory disorder, causing high morbidity and mortality risk in children and adults. It can be subdivided into a primary, genetic form and a secondary, acquired form that complicates diverse infections, malignancies and autoimmune or autoinflammatory disorders. Both subtypes present with the same spectrum of non‐specific symptoms, making accurate diagnosis and rapid treatment initiation challenging. In the last decade, increased awareness and international collaborative efforts fuelled a marked progress in diagnostic protocols and novel treatment strategies for HLH and new diagnostic guidelines are being tailored to specific secondary HLH subtypes. Therapy is gradually shifting its focus from overall immunosuppression towards targeting specific cytokines, cell types or signalling pathways underlying pathophysiology. Nevertheless, continued research efforts remain indispensable to customize therapy to individual patient needs.

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References

Details

Published: Jun 12, 2016
Vol/Issue: 174(2)
Pages: 175-187
License: View

Authors

E

Ellen Brisse

Laboratory of Immunobiology Rega Institute KU Leuven Leuven Belgium

P

Patrick Matthys

Laboratory of Immunobiology Rega Institute KU Leuven Leuven Belgium

C

Carine H. Wouters

Laboratory of Paediatric Immunology KU Leuven University Hospital Gasthuisberg Leuven Belgium

Funding

Agentschap voor Innovatie door Wetenschap en Technologie

Cite This Article

Ellen Brisse, Patrick Matthys, Carine H. Wouters (2016). Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options. British Journal of Haematology, 174(2), 175-187. https://doi.org/10.1111/bjh.14144

Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options

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