High Mortality of HLH in ICU Regardless Etiology or Treatment
Background: Adult hemophagocytic lymphohistiocytosis (HLH) is highly lethal in the ICU. The diagnostic and therapeutic emergency that HLH represents is compounded by its unknown pathophysiological mechanisms. Here, we report on a large cohort of adult HLH in the ICU (ICU-HLH). We analyzed prognostic...
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| Language: | English |
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Frontiers Media S.A.
2021-10-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2021.735796/full |
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| author | Amandine Bichon Amandine Bichon Jérémy Bourenne Jérémy Bourenne Jérôme Allardet-Servent Laurent Papazian Laurent Papazian Sami Hraiech Sami Hraiech Christophe Guervilly Christophe Guervilly Vanessa Pauly Gilles Kaplanski Gilles Kaplanski Djamel Mokart Marc Gainnier Marc Gainnier Julien Carvelli Julien Carvelli |
| author_facet | Amandine Bichon Amandine Bichon Jérémy Bourenne Jérémy Bourenne Jérôme Allardet-Servent Laurent Papazian Laurent Papazian Sami Hraiech Sami Hraiech Christophe Guervilly Christophe Guervilly Vanessa Pauly Gilles Kaplanski Gilles Kaplanski Djamel Mokart Marc Gainnier Marc Gainnier Julien Carvelli Julien Carvelli |
| author_sort | Amandine Bichon |
| collection | DOAJ |
| description | Background: Adult hemophagocytic lymphohistiocytosis (HLH) is highly lethal in the ICU. The diagnostic and therapeutic emergency that HLH represents is compounded by its unknown pathophysiological mechanisms. Here, we report on a large cohort of adult HLH in the ICU (ICU-HLH). We analyzed prognostic factors associated with mortality to define the diagnostic and therapeutic challenges in this specific population.Methods: This retrospective study included adult patients diagnosed with HLH in four ICUs in Marseille, France between 2010 and 2020. Patients who fulfilled the HLH-2004 criteria (≥ 4/8) and/or had an HScore ≥ 169 were diagnosed with HLH. HLH was categorized into four groups according to etiology: sepsis-associated HLH, intracellular infection-associated HLH, malignancy-associated HLH, and idiopathic HLH.Results: Two hundred and sixty patients were included: 121 sepsis-associated HLH (47%), 84 intracellular infection-associated HLH (32%), 28 malignancy-associated HLH (11%), and 27 idiopathic HLH (10%). The ICU mortality rate reached 57% (n = 147/260) without a statistical difference between etiological groups. Independent factors associated with mortality in multivariate analysis included age (OR (5 years) = 1.31 [1.16–1.48], p < 0.0001), SOFA score at ICU admission (OR = 1.37 [1.21–1.56], p < 0.0001), degradation of the SOFA score between ICU arrival and HLH diagnosis (Delta SOFA) (OR = 1.47 [1.28–1.70], p < 0.0001), the presence of bone-marrow hemophagocytosis (OR = 5.27 [1.11–24.97], p = 0.04), highly severe anemia (OR = 1.44 [1.09–1.91], p = 0.01), and hypofibrinogenemia (OR = 1.21 [1.04–1.41], p = 0.02).Conclusions: In this large retrospective cohort study of critically ill patients, ICU-HLH in adults was associated with a 57% mortality rate, regardless of HLH etiology or specific treatment. Factors independently associated with prognosis included age, presence of hemophagocytosis in bone-marrow aspirates, organ failure at admission, and worsening organ failure during the ICU stay. Whether a rapid diagnosis and the efficacy of specific therapy improve outcome is yet to be prospectively investigated. |
| first_indexed | 2024-12-18T00:25:57Z |
| format | Article |
| id | doaj.art-0d293fd78ba8403ab08f74212e0db624 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-12-18T00:25:57Z |
| publishDate | 2021-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-0d293fd78ba8403ab08f74212e0db6242022-12-21T21:27:15ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-10-01810.3389/fmed.2021.735796735796High Mortality of HLH in ICU Regardless Etiology or TreatmentAmandine Bichon0Amandine Bichon1Jérémy Bourenne2Jérémy Bourenne3Jérôme Allardet-Servent4Laurent Papazian5Laurent Papazian6Sami Hraiech7Sami Hraiech8Christophe Guervilly9Christophe Guervilly10Vanessa Pauly11Gilles Kaplanski12Gilles Kaplanski13Djamel Mokart14Marc Gainnier15Marc Gainnier16Julien Carvelli17Julien Carvelli18APHM, University Timone Hospital, Réanimation des Urgences, Marseille, FranceAix-Marseille University, Marseille, FranceAPHM, University Timone Hospital, Réanimation des Urgences, Marseille, FranceAix-Marseille University, Marseille, FranceDepartment of Intensive Care, Européen Hospital, Marseille, FranceAix-Marseille University, Marseille, FranceDepartment of Respiratory and Infectious Intensive Care, APHM, University Nord Hospital, Marseille, FranceAix-Marseille University, Marseille, FranceDepartment of Respiratory and Infectious Intensive Care, APHM, University Nord Hospital, Marseille, FranceAix-Marseille University, Marseille, FranceDepartment of Respiratory and Infectious Intensive Care, APHM, University Nord Hospital, Marseille, FranceDepartment of Medical Information, CEReSS - Health Service Research and Quality of Life Center, APHM, Aix-Marseille University, Marseille, FranceAix-Marseille University, Marseille, FranceDepartment of Internal Medicine and Clinical Immunology, APHM, University Conception Hospital, Marseille, FranceDepartment of Onco-Hematological Intensive Care, Paoli Calmette Institute, Marseille, FranceAPHM, University Timone Hospital, Réanimation des Urgences, Marseille, FranceAix-Marseille University, Marseille, FranceAPHM, University Timone Hospital, Réanimation des Urgences, Marseille, FranceAix-Marseille University, Marseille, FranceBackground: Adult hemophagocytic lymphohistiocytosis (HLH) is highly lethal in the ICU. The diagnostic and therapeutic emergency that HLH represents is compounded by its unknown pathophysiological mechanisms. Here, we report on a large cohort of adult HLH in the ICU (ICU-HLH). We analyzed prognostic factors associated with mortality to define the diagnostic and therapeutic challenges in this specific population.Methods: This retrospective study included adult patients diagnosed with HLH in four ICUs in Marseille, France between 2010 and 2020. Patients who fulfilled the HLH-2004 criteria (≥ 4/8) and/or had an HScore ≥ 169 were diagnosed with HLH. HLH was categorized into four groups according to etiology: sepsis-associated HLH, intracellular infection-associated HLH, malignancy-associated HLH, and idiopathic HLH.Results: Two hundred and sixty patients were included: 121 sepsis-associated HLH (47%), 84 intracellular infection-associated HLH (32%), 28 malignancy-associated HLH (11%), and 27 idiopathic HLH (10%). The ICU mortality rate reached 57% (n = 147/260) without a statistical difference between etiological groups. Independent factors associated with mortality in multivariate analysis included age (OR (5 years) = 1.31 [1.16–1.48], p < 0.0001), SOFA score at ICU admission (OR = 1.37 [1.21–1.56], p < 0.0001), degradation of the SOFA score between ICU arrival and HLH diagnosis (Delta SOFA) (OR = 1.47 [1.28–1.70], p < 0.0001), the presence of bone-marrow hemophagocytosis (OR = 5.27 [1.11–24.97], p = 0.04), highly severe anemia (OR = 1.44 [1.09–1.91], p = 0.01), and hypofibrinogenemia (OR = 1.21 [1.04–1.41], p = 0.02).Conclusions: In this large retrospective cohort study of critically ill patients, ICU-HLH in adults was associated with a 57% mortality rate, regardless of HLH etiology or specific treatment. Factors independently associated with prognosis included age, presence of hemophagocytosis in bone-marrow aspirates, organ failure at admission, and worsening organ failure during the ICU stay. Whether a rapid diagnosis and the efficacy of specific therapy improve outcome is yet to be prospectively investigated.https://www.frontiersin.org/articles/10.3389/fmed.2021.735796/fullhemophagocytic lymphohistiocytosismacrophage activation syndromehemophagocytosiscytokine stormintensive care unit |
| spellingShingle | Amandine Bichon Amandine Bichon Jérémy Bourenne Jérémy Bourenne Jérôme Allardet-Servent Laurent Papazian Laurent Papazian Sami Hraiech Sami Hraiech Christophe Guervilly Christophe Guervilly Vanessa Pauly Gilles Kaplanski Gilles Kaplanski Djamel Mokart Marc Gainnier Marc Gainnier Julien Carvelli Julien Carvelli High Mortality of HLH in ICU Regardless Etiology or Treatment Frontiers in Medicine hemophagocytic lymphohistiocytosis macrophage activation syndrome hemophagocytosis cytokine storm intensive care unit |
| title | High Mortality of HLH in ICU Regardless Etiology or Treatment |
| title_full | High Mortality of HLH in ICU Regardless Etiology or Treatment |
| title_fullStr | High Mortality of HLH in ICU Regardless Etiology or Treatment |
| title_full_unstemmed | High Mortality of HLH in ICU Regardless Etiology or Treatment |
| title_short | High Mortality of HLH in ICU Regardless Etiology or Treatment |
| title_sort | high mortality of hlh in icu regardless etiology or treatment |
| topic | hemophagocytic lymphohistiocytosis macrophage activation syndrome hemophagocytosis cytokine storm intensive care unit |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2021.735796/full |
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