Article
Critical Care Medicine
Sandrine Valade, Gregoire Monseau, Eric Mariotte, Michael Darmon
Summary: The study aimed to assess features mimicking hemophagocytic syndrome in critically ill hematologic patients using diagnostic scores. Results indicate that both HScore and hemophagocytic lymphohistiocytosis criteria are highly accurate in identifying hemophagocytic syndrome in ICU patients, despite potential confounding factors.
CRITICAL CARE MEDICINE
(2021)
Article
Immunology
France Debaugnies, Bhavna Mahadeb, Carole Nagant, Nathalie Meuleman, David De Bels, Fleur Wolff, Philippe Gottignies, Adriano Salaroli, Patricia Borde, Michel Voue, Francis Corazza
Summary: This study compared diagnostic biomarkers for HLH in critically ill adult patients and found that ferritin, IL-18, and glycosylated ferritin were the most effective parameters for early diagnosis, with IL18*HScore identified as a highly potential score.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Article
Medicine, General & Internal
D. A. Eichenauer, G. Lachmann, P. La Rosee
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome mainly found in adults, often triggered by infections, malignancies, or autoimmune disorders. Early diagnosis and timely immunosuppressive therapy are crucial for the course and prognosis of HLH patients, whose overall prognosis is generally poor.
MEDIZINISCHE KLINIK-INTENSIVMEDIZIN UND NOTFALLMEDIZIN
(2021)
Article
Critical Care Medicine
Frapard Thomas Md, Darmon Michael M. D. PhD, Fadlallah Jehane M. D. PhD, Mariotte Eric Md, Valade Sandrine Md
Summary: This study describes the characteristics of patients with the rare condition hemophagocytic syndrome, showing a high in-hospital mortality rate but significant improvement in hemodynamic parameters following etoposide infusion.
JOURNAL OF CRITICAL CARE
(2022)
Review
Critical Care Medicine
Melissa R. Hines, Tatiana von Bahr Greenwood, Gernot Beutel, Karin Beutel, J. Allyson Hays, AnnaCarin Horne, Gritta Janka, Michael B. Jordan, Jan A. M. van Laar, Gunnar Lachmann, Kai Lehmberg, Rafal Machowicz, Paivi Miettunen, Paul La Rosee, Bita Shakoory, Matt S. Zinter, Jan-Inge Henter
Summary: This article provides guidelines for the recognition, diagnosis, and treatment of hemophagocytic lymphohistiocytosis (HLH) in ICU patients. Prompt diagnosis and early aggressive treatment are crucial for managing the multisystem organ failure associated with HLH. Thorough investigation of underlying triggers is necessary. Early steroid treatment is important for familial HLH and some acquired HLH cases, and additional therapies may be needed for patients with macrophage activation syndrome.
CRITICAL CARE MEDICINE
(2022)
Article
Medicine, General & Internal
Jamie Allen, Matthew M. McCambridge, Hope Kincaid, Joshua A. Kalter
Summary: Among critically ill COVID-19 positive patients, the incidence of sHLH is higher than previously reported in patients with non-COVID-19 related sepsis. Clinicians caring for COVID-19 patients should consider this secondary diagnosis and subsequent appropriate treatments, especially in those requiring mechanical ventilation.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Multidisciplinary Sciences
Sandrine Valade, Berangere S. Joly, Agnes Veyradier, Jehane Fadlallah, Lara Zafrani, Virginie Lemiale, Amelie Launois, Alain Stepanian, Lionel Galicier, Claire Fieschi, Adrien Mirouse, Jean Jacques Tudesq, Anne-Claire Lepretre, Elie Azoulay, Michael Darmon, Eric Mariotte
Summary: Coagulation disorders are common in critically ill patients with hemophagocytic lymphohistiocytosis (HLH) and are associated with a high risk of severe bleeding and mortality. High fibrinolytic activity and decreased ADAMTS13 activity were observed in these patients, with severe bleeding events and initial SOFA score being independent predictors of hospital mortality. Targeting the plasmin system may be a promising approach in managing severe HLH-related coagulation disorders.
Review
Critical Care Medicine
Julie Helms, Toshiaki Iba, Jean Marie Connors, Satoshi Gando, Marcel Levi, Ferhat Meziani, Jerrold H. Levy
Summary: Coagulopathy is a common and severe complication among critically ill patients, with variable pathogenesis and presentation depending on the underlying disease. This review classifies coagulopathies into hemorrhagic type characterized by hypocoagulable and hyperfibrinolysis state, and thrombotic type with systemic prothrombotic and antifibrinolytic phenotype. The article discusses the differences in pathogenesis and treatment approaches for these common coagulopathies.
INTENSIVE CARE MEDICINE
(2023)
Article
Critical Care Medicine
Erik H. A. Michels, Joe A. Butler, Tom D. Y. Reijnders, Olaf Cremer, Brendon M. Scicluna, Fabrice Uhel, Hessel F. Peters-Sengers, Marcus Schultz, Julian E. Knight, Lonneke van Vught, Tom M. van der Poll
Summary: This study provides evidence that older age is associated with attenuated endothelial cell activation and dysfunction in sepsis, as well as impaired immune and cytokine signaling based on blood leukocyte transcriptome analysis. These findings suggest that age should be considered when selecting patients for sepsis trials targeting the immune system or endothelial cell response.
Article
Pharmacology & Pharmacy
Jia-Jia Huang, Zhi-Ye Zou, Zhi-Peng Zhou, Yan Liu, Zhen-Jia Yang, Jing-Jing Zhang, Ying-Yi Luan, Yong-Ming Yao, Ming Wu
Summary: This study aimed to investigate whether early unfractionated heparin (UFH) administration provides a survival advantage for patients with sepsis-induced coagulopathy (SIC). The results showed that early UFH therapy appears to be associated with improved outcomes.
FRONTIERS IN PHARMACOLOGY
(2023)
Article
Medicine, General & Internal
Laurine Diaz, Benjamin Jauzelon, Anne-Charlotte Dillies, Cosette Le Souder, Jean-Luc Faillie, Alexandre Thibault Jacques Maria, Pascale Palassin
Summary: Acquired hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal condition characterized by hyperactivation of macrophages and cytotoxic lymphocytes. Immune checkpoint inhibitors (ICI), a type of anti-tumor agents, have been associated with HLH. Our analysis found that HLH was seven times more frequently reported with ICI therapy than with other drugs. Most HLH cases had a favorable outcome, but a small percentage resulted in death.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Immunology
Esther J. Nossent, Alex R. Schuurman, Tom D. Y. Reijnders, Anno Saris, Ilse Jongerius, Siebe G. Blok, Heder de Vries, JanWillem Duitman, Anton Vonk Noordegraaf, Lilian J. Meijboom, Rene Lutter, Leo Heunks, Harm Jan Bogaard, Tom van der Poll
Summary: This study evaluated the activation of host response pathways in the lung compartment of critically ill COVID-19 patients with persistent acute respiratory distress syndrome. The results showed strong local responses relating to coagulation, the complement system, cytokines, chemokines and growth factors in the bronchoalveolar compartment, with some biomarkers declining in levels at 3 to 4 weeks after mechanical ventilation initiation.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Anesthesiology
Mirjam Bachler, Johannes Boesch, Daniel P. Stuerzel, Tobias Hell, Andreas Giebl, Mathias Stroehle, Sebastian J. Klein, Volker Schaefer, Georg F. Lehner, Michael Joannidis, Claudius Thome, Dietmar Fries
Summary: Critically ill COVID-19 patients exhibit a hypercoagulable state with impaired fibrinolysis, characterized by increased clot firmness and decreased fibrinolytic response in whole blood coagulation tests. This hypofibrinolytic state may be at least partially due to a decreased fibrinolytic response.
BRITISH JOURNAL OF ANAESTHESIA
(2021)
Review
Oncology
Claire Jing-Wen Tan, Zheng Qin Ng, Rajat Bhattacharyya, Rehena Sultana, Jan Hau Lee
Summary: This systematic review aimed to determine the mortality rate and factors associated with mortality in critically ill children with HLH. The study found that children with HLH have high mortality rates and require substantial PICU support. Collaboration between multiple centers with standardized data collection may provide more robust data for future research.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Hematology
Pascal Migaud, Markus Mueller, Keikawus Arasteh, Marcus Hentrich, Hartmut Stocker
Summary: Compared to the general population, the incidence of lymphoproliferative disorders (LPDs) is higher among people living with HIV (PLHIV). In a retrospective cohort study, 6% of HIV-infected patients with LPDs were found to have hemophagocytic lymphohistiocytosis (HLH), and HLH was associated with lower overall survival.
ANNALS OF HEMATOLOGY
(2022)
Letter
Hematology
Maxime Delrue, Minh Tam Baylatry, Anne-Christine Joly, Elise Corre, Zora Marjanovic, Noujoud El-Khoury-Hanna, Frederic Feger, Ludovic Suner, Agnes Veyradier, Alain Stepanian, Paul Coppo
AMERICAN JOURNAL OF HEMATOLOGY
(2021)
Article
Oncology
Jean-Jacques Tudesq, Sandrine Valade, Lionel Galicier, Lara Zafrani, David Boutboul, Cedric de Bazelaire, Nicolas Munoz-Bongrand, Emmanuel Canet, Fanny Ardisson, Virginie Lemiale, Michael Darmon, Elie Azoulay, Eric Mariotte
Summary: Management of reactive hemophagocytic lymphohistiocytosis (rHLH) requires early recognition, identification of triggers, and adequate treatment to reduce mortality. Tissue biopsies have a high diagnostic yield in identifying triggers in severe rHLH cases. While splenectomy carries a procedural risk, it can be reserved for cases where diagnostic uncertainty persists. Early initiation of appropriate therapy based on trigger identification leads to better outcomes in patients with rHLH.
HEMATOLOGICAL ONCOLOGY
(2021)
Article
Medicine, General & Internal
Olivier Hermine, Xavier Mariette, Pierre-Louis Tharaux, Matthieu Resche-Rigon, Raphael Porcher, Philippe Ravaud
Summary: In this clinical trial of COVID-19 patients with pneumonia requiring oxygen support but not in the ICU, tocilizumab did not reduce WHO-CPS scores below 5 by day 4 but may have reduced the risk of needing noninvasive ventilation, mechanical ventilation, or death by day 14. There was no difference in mortality by day 28. Further studies are needed to confirm these preliminary results.
JAMA INTERNAL MEDICINE
(2021)
Letter
Hematology
Pierre-Edouard Debureaux, Mathilde Ruggiu, Stephanie Mathis, Adrien Mirouse, Bedis Dhouaieb, Wendy Cuccuini, Jean Soulier, Eric Mariotte, Pierre Lemaire
ANNALS OF HEMATOLOGY
(2022)
Letter
Hematology
Julien Schmidt, Lara Zafrani, Virginie Lemiale, Alain Stepanian, Berangere Joly, Elie Azoulay, Eric Mariotte
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Critical Care Medicine
Xavier Mariette, Olivier Hermine, Pierre Louis Tharaux, Matthieu Resche-Rigon, Raphael Porcher, Philippe Ravaud, Serge Bureau, Maxime Dougados, Annick Tibi
Summary: The study aimed to determine the effectiveness of anakinra in improving outcomes of patients with mild-to-moderate COVID-19 pneumonia, but the results showed that anakinra did not show improvement in patient outcomes. Further studies are needed to assess the efficacy of anakinra in other selected groups of patients with more severe COVID-19.
LANCET RESPIRATORY MEDICINE
(2021)
Article
Critical Care Medicine
Julien Demiselle, Valerie Seegers, Marie Lemerle, Ferhat Meziani, Fabien Grelon, Bruno Megarbane, Nadia Anguel, Jean-Paul Mira, Pierre-Francois Dequin, Soizic Gergaud, Nicolas Weiss, Francois Legay, Yves Le Tulzo, Marie Conrad, Rene Robert, Frederic Gonzalez, Christophe Guitton, Fabienne Tamion, Jean-Marie Tonnelier, Jean-Pierre Bedos, Thierry Van der Linden, Antoine Vieillard-Baron, Eric Mariotte, Gael Pradel, Olivier Lesieur, Jean-Damien Ricard, Fabien Herve, Damien du Cheyron, Claude Guerin, Jean-Louis Teboul, Julie Helms, Peter Radermacher, Pierre Asfar
Summary: The SEPSISPAM trial found that targeting high mean arterial pressure may reduce acute kidney injury in septic shock patients with chronic hypertension, and post hoc analysis showed that chronic hypertensive patients treated with angiotensin II receptor blocker might benefit from a high mean arterial pressure target to reduce the risk of acute kidney injury.
CRITICAL CARE MEDICINE
(2021)
Article
Hematology
Eric Mariotte, Lara Zafrani, Jehane Fadlallah, Lionel Galicier, Etienne Ghrenassia, Lionel Kerhuel, Laure Calvet, Audrey De Jong, Virginie Lemiale, Sandrine Valade, Berangere S. Joly, Alain Stepanian, Elie Azoulay, Michael Darmon
Summary: This study evaluated the accuracy of diagnostic scores for thrombotic thrombocytopenic purpura (TTP) in an intensive care unit (ICU) setting, finding that platelet count could be similarly used to differentiate TTP from non-TTP patients as the Coppo score.
THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Virology
Ali Bektas, Michael F. Covington, Guy Aidelberg, Anibal Arce, Tamara Matute, Isaac Nunez, Julia Walsh, David Boutboul, Constance Delaugerre, Ariel B. Lindner, Fernan Federici, Anitha D. Jayaprakash
Summary: The COVID-19 pandemic has highlighted the challenges in large-scale testing, leading to the development of a DIY workflow for rapid and accurate detection of viral RNA with high sensitivity and specificity. This method improves efficiency and accuracy of testing through various techniques, allowing for quick testing at home or in healthcare settings.
Article
Microbiology
Souheil Zayet, Pierre Isnard, Jacinta Bustamante, David Boutboul, Sarra Abroug, Nabil Belfeki
Summary: Whipple's Disease is a rare systemic infectious disease caused by the ubiquitous actinomycetes Tropheryma whipplei. This report describes a rare case of cutaneous granulomatosis with hypercalcemia as an unusual presenting feature of Whipple's disease. Treatment with hydroxychloroquine and doxycycline resulted in rapid resolution of symptoms and complete recovery.
Article
Immunology
Akihiro Hoshino, David Boutboul, Yuan Zhang, Hye Sun Kuehn, Jerome Hadjadj, Nihal Ozdemir, Tiraje Celkan, Christoph Walz, Capucine Picard, Christelle Lenoir, Nizar Mahlaoui, Christoph Klein, Xiao Peng, Antoine Azar, Erin Reigh, Morgane Cheminant, Alain Fischer, Frederic Rieux-Laucat, Isabelle Callebaut, Fabian Hauck, Joshua Milner, Sergio D. Rosenzweig, Sylvain Latour
Summary: This study identified IKZF1 variants in the DNA binding domain that cause inflammatory, autoimmune, allergic symptoms, and abnormal plasma cell proliferation. These variants act as gain-of-function alleles, leading to increased gene expression associated with T(H)2 and plasma cell differentiation. Treatment with Lenalidomide can correct these abnormalities.
SCIENCE IMMUNOLOGY
(2022)
Letter
Hematology
Remi Bertinchamp, Margaux Garzaro, David Boutboul, Lionel Galicier, Veronique Meignin, Julien Calvani, Mohammed El Hadi Djerad, Willy Rozenbaum, Philippe Simon, Eric Oksenhendler, Laurence Gerard
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Camille Rasmussen, Laurence Gerard, Jehane Fadlallah, Emilie Corvilain, Lionel Galicier, Veronique Meignin, Eric Oksenhendler, David Boutboul
Summary: This retrospective study analyzed the treatment outcome of 99 HHV8(+) MCD patients with rituximab-based therapy. The study found that HIV- status, high HHV8 viral load, and elevated serum CRP levels were independent risk factors for disease progression after rituximab-induced complete remission. The study also suggested that HIV+ patients had a lower progression rate, possibly due to immune restoration upon antiretroviral therapy.
Editorial Material
Clinical Neurology
Cyrille Coustal, Radjiv Goulabchand, Pierre Labauge, Philippe Guilpain, Clarisse Carra-Dalliere, Edouard Januel, Eric Jeziorski, Valery Salle, Jean-Francois Viallard, David Boutboul, Claire Fieschi, Delphine Gobert, Nathalie Aladjidi, Patricia Rullier, Julie Graveleau, Marie Piel-Julian, Felipe Suarez, Benedicte Neven, Nizar Mahlaoui, Xavier Ayrignac
Summary: Neurologic involvement is common among patients with CTLA4 deficiency, with symptoms such as headaches, focal deficit, and seizures. MRI results show contrast-enhancing lesions reminiscent of multiple sclerosis. Abatacept treatment has shown good clinical and radiologic response in these patients.