Recommendations for the management of hemophagocytic lymphohistiocytosis in adults
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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.
In: BLOOD, Vol. 133, No. 23, 06.06.2019, p. 2465-2477.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Recommendations for the management of hemophagocytic lymphohistiocytosis in adults
AU - La Rosée, Paul
AU - Horne, AnnaCarin
AU - Hines, Melissa
AU - von Bahr Greenwood, Tatiana
AU - Machowicz, Rafal
AU - Berliner, Nancy
AU - Birndt, Sebastian
AU - Gil-Herrera, Juana
AU - Girschikofsky, Michael
AU - Jordan, Michael B
AU - Kumar, Ashish
AU - van Laar, Jan A M
AU - Lachmann, Gunnar
AU - Nichols, Kim E
AU - Ramanan, Athimalaipet V
AU - Wang, Yini
AU - Wang, Zhao
AU - Janka, Gritta
AU - Henter, Jan-Inge
N1 - © 2019 by The American Society of Hematology.
PY - 2019/6/6
Y1 - 2019/6/6
N2 - 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.
AB - 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.
KW - Adult
KW - Female
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/diagnosis
KW - Male
U2 - 10.1182/blood.2018894618
DO - 10.1182/blood.2018894618
M3 - SCORING: Review article
C2 - 30992265
VL - 133
SP - 2465
EP - 2477
JO - BLOOD
JF - BLOOD
SN - 0006-4971
IS - 23
ER -
