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Cited by 1,108

The DOI matches its Crossref record; 108 of the 108 references it cites were found in a registry or an index.

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

La Rosée, Paul, Horne, AnnaCarin, Hines, Melissa, von Bahr Greenwood, Tatiana, Machowicz, Rafal, Berliner, Nancy, Birndt, Sebastian, Gil-Herrera, Juana, Girschikofsky, Michael, Jordan, Michael B., Kumar, Ashish, van Laar, Jan A. M., Lachmann, Gunnar, Nichols, Kim E., Ramanan, Athimalaipet V., Wang, Yini, Wang, Zhao, Janka, Gritta, Henter, Jan-Inge·2019·Blood

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, whereas the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS; or MAS-HLH). Most information on the diagnosis and treatment of HLH comes from the pediatric literature. Although helpful in some adult cases, this raises several challenges. For example, the HLH-2004 diagnostic criteria developed for children are commonly applied but are not validated for adults. Another challenge in HLH diagnosis is that patients may present with a phenotype indistinguishable from sepsis or multiple organ dysfunction syndrome. Treatment algorithms targeting hyperinflammation are frequently based on pediatric protocols, such as HLH-94 and HLH-2004, which may result in overtreatment and unnecessary toxicity in adults. Therefore, dose reductions, individualized tailoring of treatment duration, and an age-dependent modified diagnostic approach are to be considered. Here, we present expert opinions derived from an interdisciplinary working group on adult HLH, sponsored by the Histiocyte Society, to facilitate knowledge transfer between physicians caring for pediatric and adult patients with HLH, with the aim to improve the outcome for adult patients affected by HLH.

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Metadata

Authors
La Rosée, P., Horne, A., Hines, M., von Bahr Greenwood, T., Machowicz, R., Berliner, N., Birndt, S., Gil-Herrera, J., Girschikofsky, M., Jordan, M. B., Kumar, A., van Laar, J. A. M., Lachmann, G., Nichols, K. E., Ramanan, A. V., Wang, Y., Wang, Z., Janka, G., & Henter, J.-I.
Venue
Blood
Published
2019-06-06
Volume
133
Issue
23
Pages
2465-2477
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
OpenAlex
W2936019126
Cited by
1,108
References
108
FWCI
73.42
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. https://app.cochat.ai/papers/10.1182/blood.2018894618
The paper
La Rosée, P., Horne, A., Hines, M., von Bahr Greenwood, T., Machowicz, R., Berliner, N., Birndt, S., Gil-Herrera, J., Girschikofsky, M., Jordan, M. B., Kumar, A., van Laar, J. A. M., Lachmann, G., Nichols, K. E., Ramanan, A. V., Wang, Y., Wang, Z., Janka, G., & Henter, J.-I. (2019). Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. https://doi.org/10.1182/blood.2018894618

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.