Association syndrome lymphohystiocytaire (HLH) et lupus systémique : étude multicentrique française de 103 épisodes chez 89 patients adultes

Gavand, Pierre-Edouard; Serio, Ilaria; Larroche, Claire; Costédoat-Chalumeau, Nathalie; Lavigne, Christian; Mouthon, Luc; Artifoni, Mathieu; Dossier, Antoine; Bloch-Queyrat, Coralie; Arnaud, Lucie; Amoura, Zahir; Martin, T.

doi:10.1016/j.revmed.2016.10.106

Cited by 3 publications

(2 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Also, patients with HLH associated with lymphomas have a higher mortality than if the underlying etiology is an infection or autoimmune condition [6]. Elevated LDH, AST, c-reactive protein, ferritin, and procalcitonin were also reported by Gavand et al to be related to poor prognosis [6,11]. Our patient is a male who presented with lymphocytopenia, increased bilirubin, BUN, AST, ferritin, and procalcitonin, which are all characteristics of a poor prognosis.…”

Section: Discussionsupporting

confidence: 62%

Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, Hodgkin’s lymphoma, and Epstein–Barr virus

Chelsea¹,

Christopher²

2019

ijcrioncology

View full text Add to dashboard Cite

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome with high rates of mortality. Secondary HLH is acquired from an underlying illness and therefore difficult to diagnosis because the underlying disease can mask the symptoms of HLH or have a similar presentation. Therefore, it is important for physicians and health care providers to be familiar with HLH to keep in mind as a differential diagnosis in critically ill patients. It is imperative to start treatment early to decrease mortality. Case Report: We describe a case of a 42-year-old African-American man with a past medical history of human immunodeficiency virus (HIV), Hodgkin's lymphoma, and Epstein-Barr virus (EBV) who presented to the emergency department (ED) with diarrhea, nausea, and vomiting. He was ultimately diagnosed with acquired HLH. Early on during his admission he was treated with dexamethasone, but continued into multiple organ failure and succumbed on the 11th hospital day. Conclusion: Hemophagocytic lymphohistiocytosis is a rare disease and may not initially or come to mind at all as part of a differential diagnosis in critically ill patients.

show abstract

Section: Discussionsupporting

confidence: 62%

Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, Hodgkin’s lymphoma, and Epstein–Barr virus

Chelsea¹,

Christopher²

2019

ijcrioncology

View full text Add to dashboard Cite

show abstract

“…In the cases presented here, case 1 and 2’s clinical onset of HLH did coincide with the new onset of SLE and does fulfill the SLICC criteria for SLE. Amongst all cases of SLE and HLH, including rheumatologic flares, the incidence of HLH is approximately 0.9–4.6% [13]. As has been described by several groups, the difficulty in making the diagnosis of HLH in new onset SLE arise from the overlap of many symptoms, making the use of the HLH-2004 criteria critical for acute and timely diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature

et al. 2019

View full text Add to dashboard Cite

Background Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population. HLH has been associated with infections, malignancy and autoimmune conditions such as Systemic Lupus Erythematosus (SLE), however this is often in the context of a disease flare. Currently, there are limited reports of inaugural SLE manifesting as HLH with a lack of consensus on treatment and management of these patients. Case presentation Here, we present two rare case reports of severe cutaneous manifestation of lupus associated with HLH. Both patients presented with sinister clinical courses with primarily rheumatologic complaints including malaise, arthralgia, and myalgia with biochemical abnormalities. Both patients were diagnosed with HLH as a result of first presentation from cutaneous lupus. A comprehensive literature review using the PubMed database with cases comprising keywords of HLH and SLE up to September 2017 was conducted, with an emphasis on inaugural cutaneous SLE cases. Conclusions Ultimately, we highlight that a keen clinical acumen is required as misdiagnosis may lead to insufficient treatment with adverse clinical outcomes with the unique presentation of HLH from inaugural cases of SLE.

show abstract

Hemophagocytic Lymphohistiocytosis Unmasking Systemic Lupus Erythematosus: Management With Belimumab and a Case Study

Ahmad,

Atluri,

Robbins

2024

Cureus

View full text Add to dashboard Cite

Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition caused by the hyperactivation of macrophages and T-cells, triggered by infection, malignancy, or underlying rheumatological conditions. It rarely presents as a first manifestation of a rheumatological condition. Macrophage activation syndrome (MAS) is secondary HLH associated with underlying hematological conditions. Here, we present a case of a previously healthy 29-year-old female who was admitted with fever, rash, and pancytopenia, found to have HLH, and a workup revealed underlying systemic lupus erythematosus (SLE). She was successfully treated with dexamethasone, etoposide, and belimumab, with complete recovery of her symptoms. This case highlights the importance of a thorough evaluation of rheumatological conditions in all patients with HLH despite their previous medical history and the use of belimumab for SLE.

show abstract

Association syndrome lymphohystiocytaire (HLH) et lupus systémique : étude multicentrique française de 103 épisodes chez 89 patients adultes

Cited by 3 publications

References 0 publications

Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, Hodgkin’s lymphoma, and Epstein–Barr virus

Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, Hodgkin’s lymphoma, and Epstein–Barr virus

Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature

Hemophagocytic Lymphohistiocytosis Unmasking Systemic Lupus Erythematosus: Management With Belimumab and a Case Study

Contact Info

Product

Resources

About