Emapalumab for adult and pediatric patients with hemophagocytic lymphohistiocytosis

Garonzi, Chiara; Chinello, Matteo; Cesaro, Simone

doi:10.1080/17512433.2021.1901576

Cited by 8 publications

(6 citation statements)

References 53 publications

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“…As a consequence of prolonged dexamethasone use, she developed myopathy, hyperglycemia requiring insulin, thrush, and significant weight gain with Cushingoid features. Emapalumab was initiated as a steroid‐sparing agent, at a dose of 1 mg/kg twice weekly, etoposide was discontinued, and corticosteroids were tapered to physiologic dosing [ 15 ]. She tolerated steroid taper without recurrence of active HLH.…”

Section: Casementioning

confidence: 99%

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Greenmyer

Wyatt

Milanovich

et al. 2022

eJHaem

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Coronavirus disease 2019 (COVID‐19) infection causes a variety of extrapulmonary complications in pediatric patients. Multisystem inflammatory syndrome and hemophagocytic lymphohistiocytosis (HLH) are related to hypercytokinemia in COVID‐19 patients. HLH is a disorder of exaggerated inflammation resulting in a cytokine storm and unrestricted hemophagocytosis. HLH can be primary (familial) or secondary (acquired). Secondary HLH (sHLH) can occur in patients with rheumatologic, oncologic, or infectious diseases. The link between COVID‐19 and HLH has been reported in pediatric patients. Here we report a case of a pediatric patient who developed refractory sHLH secondary to COVID‐19 infection and required a hematopoietic cell transplant for the cure.

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Section: Casementioning

confidence: 99%

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Greenmyer

Wyatt

Milanovich

et al. 2022

eJHaem

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“…A dose of 2,400 mg/day as a continuous IV infusion was required in a cohort of critically ill adults with MAS (14,15). Emapalumab, an IFNγ-blocking monoclonal antibody, is effective in the treatment of pHLH, although its use in sHLH/MAS has yet to be approved and is under investigation (16)(17)(18). INFγ and IFNγrelated chemokines (CXCL9, CXCL10, CXLC11) levels are notably higher in patients with active sHLH/MAS and are tightly associated with laboratory markers of disease severity (18)(19)(20).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Refractory macrophage activation syndrome requiring high-dose anakinra, emapalumab, and etoposide therapy in early-onset systemic juvenile idiopathic arthritis associated with adenoviremia

Slaney,

Modica,

Woolnough

et al. 2024

Front. Pediatr.

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Macrophage activation syndrome (MAS) is a life-threatening condition characterized by the excessive stimulation of macrophages and T lymphocytes, provoked by infections, malignancy, and autoimmune or autoinflammatory conditions such as systemic juvenile idiopathic arthritis (sJIA). Clinical signs of sJIA may include high-spiking, quotidian fevers, lymphadenopathy, hepatosplenomegaly, and a salmon-colored migratory, evanescent rash. By contrast, MAS is characterized by unremitting fevers and diffuse, fixed, maculopapular rashes. In addition to hepatosplenomegaly and lymphadenopathy, patients with MAS may also have clinical signs of coagulopathy, as well as cardiac, lung, renal, and central nervous system dysfunction. The empiric treatment for MAS is initially high-dose IV corticosteroids, but usually requires addition of immunomodulators such as tacrolimus or a biologic such as Anakinra to control. The addition of immunotherapies for MAS has improved patient outcomes. We present a 2-year-old male patient with a history of early-onset sJIA, who presented with MAS refractory to corticosteroids and anakinra triggered by adenoviremia that required addition of emapalumab to control. We believe this is the first reported case of a combination of immunosuppressive therapy of emapalumab, etoposide, anakinra, tacrolimus, and corticosteroids used in the successful treatment of infection-induced MAS in early-onset sJIA. Given the lack of treatment guidelines and approved therapies for MAS, alternative strategies should be considered for patients with an intractable course.

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“…Relapse is treated with the reintroduction of initial therapy [ 6 ]. New treatment options for the relapsing or refractory disease include emapalumab, a monoclonal antibody that inhibits interferon-γ (IFN-γ) modulating cytokine overproduction [ 10 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis and Clostridium difficile Infection: A Diagnostic Dilemma

Boldig¹,

Kiamos²,

Agrawal³

et al. 2023

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition that often goes underdiagnosed because of broad and non-specific symptomatology, usually consisting of fever, hepatosplenomegaly, and multiorgan failure. This disorder can be driven by genetic components (primary) or acquired (secondary) causes related to infectious, autoimmune, or malignant processes. HLH pathogenesis derives from overactive and dysregulated immune system responses. This disorder often goes misdiagnosed because of similar clinical and laboratory findings to septicemia. Cases of HLH most commonly coexist with Epstein-Barr virus (EBV). Clostridium difficile (C. difficile) infection causing HLH has also rarely been described in the literature. A firm knowledge of HLH association with clostridial infection is essential to recognize. A presumed diagnosis of HLH in a decompensating patient may prompt the initiation of appropriate treatment earlier and improve clinical outcomes. We discuss the diagnostic and management difficulties associated with these concurrent conditions.

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Emapalumab for adult and pediatric patients with hemophagocytic lymphohistiocytosis

Cited by 8 publications

References 53 publications

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Case Report: Refractory macrophage activation syndrome requiring high-dose anakinra, emapalumab, and etoposide therapy in early-onset systemic juvenile idiopathic arthritis associated with adenoviremia

Hemophagocytic Lymphohistiocytosis and Clostridium difficile Infection: A Diagnostic Dilemma

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