Hemophagocytic Lymphohistiocytosis in Intensive Care Unit

Barba, Thomas; Maucort-Boulch, Delphine; Iwaz, Jean; Bohé, Julien; Ninet, J.; Hot, A.; Lega, Jean‐Christophe; Guérin, Claude; Argaud, Laurent; Broussolle, C.; Jamilloux, Yvan; Richard, Jean-Christophe; Sève, P.

doi:10.1097/md.0000000000002318

Cited by 52 publications

(88 citation statements)

References 19 publications

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“…Previous studies also reported an inferior OS of patients with LAHS. Barba et al (26) demonstrated that lymphoma was one of the factors associated with increased mortality in patients with HPS. Tong et al (19) conducted a study of 28 patients with aggressive T-cell LAHS and indicated that the median survival time of the patients was 40 days.…”

Section: Discussionmentioning

confidence: 99%

Lymphoma associated hemophagocytic syndrome: A single‑center retrospective study

Chang

Cui

et al. 2018

Oncol Lett

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To improve the understanding of lymphoma associated hemophagocytic syndrome (LAHS) and find an effective treatment for this fatal disease, 57 patients with LAHS were retrospectively reviewed. The most common histopathological type was extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) (45.61%). Patients with B-cell LAHS were significantly older (P<0.001), and exhibited a higher triglyceride level (P=0.012), lower serum ferritin level (P=0.014) and lower plasma Epstein-Barr virus DNA (P<0.001) compared with patients with T/NK-cell LAHS. The median survival time of all patients was 43 days, and patients with B-cell (n=14) and T/NK-cell (n=43) LAHS had a median survival time of 55 and 40 days, respectively (P=0.797). Compared with patients who were treated based on HLH-2004 protocols combined with multidrug chemotherapy, those without chemotherapy had a reduced prognosis (P=0.002). The patients that underwent hematopoietic stem cell transplantation (HSCT) following chemotherapy had a significantly improved overall survival (OS) compared with patients that did not undergo HSCT (P=0.001). Patients with B-cell LAHS treated with rituximab (P=0.015) and patients with ENKL treated with L-asparaginase/pegaspargase (L-asp/peg) (P=0.009) had an improved prognosis compared with patients not treated with these drugs. In the T/NK-cell LAHS group, patients treated with chemotherapy containing gemcitabine did not exhibit an improved OS compared with those not treated with gemcitabine (P=0.326). Furthermore, multivariate analysis demonstrated that long diagnosis time and poor performance status were independent prognosis factors for all patients with LAHS. The present study indicated that survival time does not differ between patients with B-cell LAHS and patients with T/NK-cell LAHS. Early diagnosis and appropriate immunochemotherapy plus HSCT are essential to achieve improved outcomes. The outcome of patients with B-cell LAHS may be significantly improved following treatment with rituximab. L-asp/peg-containing regimens are promising treatments for patients with NK/T-cell LAHS.

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Section: Discussionmentioning

confidence: 99%

Lymphoma associated hemophagocytic syndrome: A single‑center retrospective study

Chang

Cui

et al. 2018

Oncol Lett

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“…Die genaue Inzidenz ist nicht bekannt, wird jedoch höher geschätzt als bisher angenommen [4]. Die Letalität ist trotz spezifischer immunsuppressiver Behandlung weiterhin sehr hoch [5]. Generell wird eine genetisch bedingte, sehr gut untersuchte Form bei Kindern von einer durch Triggererkrankungen ausgelösten Form bei Erwachsenen unterschieden [6].…”

Section: Cne Schwerpunkt | Update Sepsisunclassified

“…Die Hyperinflammation resultiert in rezidivierenden Fieberepisoden, Tachykardie, hohen Triglyzeridspiegeln, disseminierter intravasaler Gerinnung (DIC), Hemmung der Hämatopoiese und Endorganschäden mit Multiorganversagen [19] [20]. Nicht selten kommt es dabei zu neurologischen Defiziten, Lungen-, Nieren-, Leber-und/oder Kreislaufversagen [5]. Die Infiltration in Milz und Leber führt häufig zur Hepatosplenomegalie, im Knochenmark zu lebensbedrohlichen Zytopenien mit folglich schweren Blutungen und/oder Sepsis [21].…”

Section: Pathogenese Und Klinisches Bildunclassified

“…Durch die Makrophagenaktivierung kommt es zur Ausschüttung hoher Mengen von Ferritin [22]. In den meisten Fällen ist das klinische Bild der Sepsis sehr ähnlich, wobei einzelne Symptome unterschiedlich stark ausgeprägt sein können [5].…”

Section: Pathogenese Und Klinisches Bildunclassified

“…Erste Hinweise, die den Verdacht äußern lassen, sind Fieber, Zytopenien und hohe Ferritinwerte. Eine Spleno-und/ oder Hepatomegalie kann ebenfalls vorhanden sein[5].Vereinfachte Darstellung der Pathophysiologie (Quelle: Lachmann G, La Rosee P, Schenk T et al [Hemophagocytic lymphohistiocytosis : A diagnostic challenge on the ICU]. Anaesthesist 2016; 65: 776-86).…”

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Hämophagozytische Lymphohistiozytose – eine seltene, aber schwere Komplikation

Lachmann¹,

Brunkhorst²

2019

intensiv

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ZusammenfassungPatienten mit Multiorganversagen oder Sepsis, die nur unzureichend auf eine antiinfektive Therapie ansprechen oder bei denen keine infektiöse Ursache erkennbar ist, könnten als Ursache das Hyperinflammationssyndrom Hämophagozytische Lymphohistiozytose entwickelt haben. Dieses syndromale Krankheitsbild verläuft klinisch ähnlich der Sepsis und endet aufgrund der verzögerten Diagnosestellung mit irreversiblem Organversagen oft letal, wobei die Erkrankung auch im Rahmen einer Sepsis entstehen kann. Die Kenntnis dieses Syndroms ist für das Überleben der Patienten von herausragender Bedeutung.

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The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

Shakoory,

Geerlinks,

Wilejto

et al. 2023

Arthritis & Rheumatology

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ObjectiveHaemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life‐threatening systemic hyperinflammatory syndromes that can develop in most inflammatory contexts. They can progress rapidly, and early identification and management are critical for preventing organ failure and mortality. This effort aimed to develop evidence‐based and consensus‐based points to consider to assist clinicians in optimising decision‐making in the early stages of diagnosis, treatment and monitoring of HLH/MAS.MethodsA multinational, multidisciplinary task force of physician experts, including adult and paediatric rheumatologists, haematologist/oncologists, immunologists, infectious disease specialists, intensivists, allied healthcare professionals and patients/parents, formulated relevant research questions and conducted a systematic literature review (SLR). Delphi methodology, informed by SLR results and questionnaires of experts, was used to generate statements aimed at assisting early decision‐making and optimising the initial care of patients with HLH/MAS.ResultsThe task force developed 6 overarching statements and 24 specific points to consider relevant to early recognition of HLH/MAS, diagnostic approaches, initial management and monitoring of HLH/MAS. Major themes included the simultaneous need for prompt syndrome recognition, systematic evaluation of underlying contributors, early intervention targeting both hyperinflammation and likely contributors, careful monitoring for progression/complications and expert multidisciplinary assistance.ConclusionThese 2022 EULAR/American College of Rheumatology points to consider provide up‐to‐date guidance, based on the best available published data and expert opinion. They are meant to help guide the initial evaluation, management and monitoring of patients with HLH/MAS in order to halt disease progression and prevent life‐threatening immunopathology.

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Hemophagocytic Lymphohistiocytosis in Intensive Care Unit

Cited by 52 publications

References 19 publications

Lymphoma associated hemophagocytic syndrome: A single‑center retrospective study

Lymphoma associated hemophagocytic syndrome: A single‑center retrospective study

Hämophagozytische Lymphohistiozytose – eine seltene, aber schwere Komplikation

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

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