Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

Créput, Caroline; Galicier, Lionel; Buyse, Sophie; Azoulay, Élie

doi:10.1007/978-3-642-28233-1_21

Cited by 30 publications

(56 citation statements)

References 86 publications

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“…Hemophagocytic syndrome is a complex disease that may affect a number of different organs, so signs and symptoms may be extremely variable () and no finding is pathognomonic for the disease. In this context, different sets of criteria have been proposed for helping clinicians to diagnose the syndrome ().…”

Section: Discussionmentioning

confidence: 99%

“…Its cardinal features are fever, hepatosplenomegaly, pancytopenia, and widespread histiocytic tissue infiltration. There are 2 major forms of hemophagocytic syndrome: a primary (hereditary) form which occurs in early childhood, and a secondary (reactive) form which occurs at any age and is probably much more frequent than the primary form ().…”

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confidence: 99%

“…Reactive hemophagocytic syndrome may be related to infection, malignancy, or autoimmune disease (), mainly in the setting of underlying immunosuppression. It is a severe, life‐threatening condition with a mortality rate ranging from 10% in children with systemic juvenile idiopathic arthritis to 20–60% in adults with malignancy‐associated hemophagocytic syndrome (). Timely diagnosis is essential since early administration of effective therapy (e.g., etoposide) may improve survival ().…”

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confidence: 99%

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Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Fardet

Galicier

Lambotte

et al. 2014

Arthritis & Rheumatology

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Objective. Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.Methods. A multicenter retrospective cohort of 312 patients who were judged by experts to have reactive hemophagocytic syndrome (n ‫؍‬ 162), were judged by experts to not have reactive hemophagocytic syndrome (n ‫؍‬ 104), or in whom the diagnosis of reactive hemophagocytic syndrome was undetermined (n ‫؍‬ 46) was used to construct and validate the reactive hemophagocytic syndrome diagnostic score, called the HScore. Ten explanatory variables were evaluated for their association with the diagnosis of hemophagocytic syndrome, and logistic regression was used to calculate the weight of each criterion included in the score. Performance of the score was assessed using developmental and validation data sets.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Fardet

Galicier

Lambotte

et al. 2014

Arthritis & Rheumatology

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1,058

897

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“…Prognosis assessment remains challenging. Haemophagocytic syndrome may develop as a result of a strong immunological activation of natural killer (NK) cells and cytotoxic T cells and may lead to multiple organ failure (Creput et al , ; Buyse et al , ). Highly stimulated but unregulated, and often ineffective, response of lymphocytes and/or histiocytes occurs, leading to cytokine overproduction and haemophagocytosis.…”

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confidence: 99%

Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide

Arca¹,

et al. 2014

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Reactive haemophagocytic syndrome is a life-threatening disease for which factors influencing the outcome remain unclear. We sought to identify determinants of early mortality in patients with reactive haemophagocytic syndrome by conducting a non-interventional retrospective multicentre study in three tertiary care teaching hospitals over a 6-year period. The medical files of 162 patients fulfilling our diagnostic criteria of haemophagocytic syndrome were reviewed. Patients were classified according to 30-d outcome following diagnosis. Thirty-three patients (20·4%) died within 30 d. Clinical features at diagnosis associated with 30-d death in univariate analysis were older age (P = 0·004), underlying lymphoma (P = 0·04), lower platelet count (P = 0·001) and elevated aspartate aminotransferase and lactate dehydrogenase (P = 0·04 both). The use of etoposide as a first-line treatment tended to be associated with a better outcome (P = 0·079). In multivariate analyses, increasing age, decreasing platelet count, underlying lymphoma and no etoposide in the management were associated with a poorer prognosis (P = 0·03, 0·01, 0·003 and 0·04, respectively). These prognostic factors could help to identify those patients more severely affected by reactive haemophagocytic syndrome, who should benefit from aggressive supportive care, combined with specific treatment of the precipitating factor.

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“…Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of dysregulatory activation of the immune system and often leads to progressive multiple organ failure . The major barrier to initiating treatment for HLH is misdiagnosis or delayed diagnosis, which is partly attributed to the rarity of HLH, its variable presentation, and the time required to perform diagnostic testing .…”

Section: Introductionmentioning

confidence: 99%

Risk of hemophagocytic lymphohistiocytosis in adults with fevers of unknown origin: the clinical utility of a new scoring system on early detection

Wang

Yang

Chiou

et al. 2016

Hematological Oncology

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The diagnosis of hemophagocytic lymphohistiocytosis (HLH) is delayed by most physicians. This study aimed to identify early parameters and suitable scoring systems for the risk of HLH. Clinical and laboratory data collected ≤3 days after admission were defined as early parameters and used to calculate the number of HLH-2004 criteria met and bone marrow (BM) score. Between January 2006 and February 2016, 233 immunocompetent adults with naïve fever of unknown origin who underwent a BM study were enrolled to mimic patients at risk of HLH and randomly assigned into the developmental or validation cohort. Hemophagocytic lymphohistiocytosis was finally diagnosed in 47 patients, with non-Hodgkin lymphoma as the major etiology (51.1%). Upon admission, four-fifths of patients who developed subsequent HLH fulfilled ≤3 of 8 HLH-2004 criteria, and 6 early parameters were independent predictors of HLH: anemia (hemoglobin < 10 g/dL), thrombocytopenia (platelet count < 100 × 10 /μL), leukoerythroblastosis, hyperbilirubinemia (total bilirubin > 2 × upper normal limit), hyperferritinemia (ferritin > 1000 ng/mL), and splenomegaly. Compared with the HLH criteria met upon admission, the BM score was an independent predictor (odds ratio = 1.621; 95% confidence interval, 1.355-1.940) with excellent discrimination (area under the receiver operating characteristic curve = 0.920; 95% confidence interval, 0.883-0.958). The sensitivity and specificity for a BM score cutoff of 10 points were 95% and 75%, respectively. When approaching immunocompetent adults with a continuously high fever, the BM score at initial admission assists with early identification of patients at risk of HLH.

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Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

Cited by 30 publications

References 86 publications

Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide

Risk of hemophagocytic lymphohistiocytosis in adults with fevers of unknown origin: the clinical utility of a new scoring system on early detection

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