Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases

Baverez, C.; Grall, Maximilien; Gerfaud‐Valentin, Mathieu; Gail, S. De; Belot, Alexandre; Perpoint, Thomas; Weber, Edward C.; Reynaud, Quitterie; Sève, P.; Jamilloux, Yvan

doi:10.3390/jcm11195799

Cited by 14 publications

(12 citation statements)

References 13 publications

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“…The utilization of anakinra for treatment of secondary HLH has been previously described in some small series of pediatric patients; however, its use in adults has only recently been suggested to provide therapeutic benefit. [2][3][4]22,23 In a case series of 21 patients (18 adults), anakinra was predominantly dosed at 100 mg/day, and yielded a response and relapse rate of 90.5% and 21.1%, respectively. 22 In another case series of 16 adult patients who received a median daily dose of anakinra 350 mg, the author found that survival benefit was demonstrated only in rheumatologic-associated HLH over other underlying etiologies (75% vs. 17%; p = .029).…”

Section: Immune Dysregulation Associated With Hlh Results In Signific...mentioning

confidence: 99%

“…[2][3][4]22,23 In a case series of 21 patients (18 adults), anakinra was predominantly dosed at 100 mg/day, and yielded a response and relapse rate of 90.5% and 21.1%, respectively. 22 In another case series of 16 adult patients who received a median daily dose of anakinra 350 mg, the author found that survival benefit was demonstrated only in rheumatologic-associated HLH over other underlying etiologies (75% vs. 17%; p = .029). 23 In our cohort, anakinra was dosed at 100 mg/day for the majority of patients, which translates to a mean ± SD anakinra dosage of 1.4 ± 0.5 mg/kg/day.…”

Section: Immune Dysregulation Associated With Hlh Results In Signific...mentioning

confidence: 99%

“…No relapses were experienced in patients who achieved responses with anakinra and overall survival at 1 year was the highest compared to the HLH‐94 protocol, HDS alone, and supportive care. The utilization of anakinra for treatment of secondary HLH has been previously described in some small series of pediatric patients; however, its use in adults has only recently been suggested to provide therapeutic benefit 2–4,22,23 . In a case series of 21 patients (18 adults), anakinra was predominantly dosed at 100 mg/day, and yielded a response and relapse rate of 90.5% and 21.1%, respectively 22 .…”

Section: Discussionmentioning

confidence: 99%

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Anakinra versus etoposide‐based therapy added to high‐dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis

Lee,

Cao,

Vittayawacharin

et al. 2023

European J of Haematology

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Objective Hemophagocytic lymphohistiocytosis (HLH) is a rare life‐threatening, hyperinflammatory syndrome usually treated with high‐dose steroids (HDS), often complemented with adjunct therapies, such as etoposide (HLH‐94 protocol). Anakinra has been reported to effectively treat HLH; however, has not been comparatively examined with etoposide‐based therapies. We sought to evaluate the effectiveness and durability of these treatment approaches. Methods We performed a retrospective analysis of all adult patients diagnosed with secondary HLH between January 2011 and November 2022 who received anakinra and HDS, the HLH‐94 protocol, HDS alone, or supportive care. Results Thirty adult patients with secondary HLH were included. Cumulative incidence (CI) of response at 30 days was 83.3%, 60%, and 36.4% for patients treated with anakinra, the HLH‐94 protocol, and HDS alone, respectively. CI of relapse at 1 year was 50%, 33.3%, and 0% with the HLH‐94 protocol, HDS, and anakinra and HDS, respectively. Overall survival at 1 year was higher with anakinra and HDS compared to the HLH‐94 protocol, yet was not statistically significant (77.8% vs. 33.3%; hazard ratio: 0.29; p = .25). Conclusion Treatment with anakinra and HDS in adults with secondary HLH was associated with higher response rates with longer survival compared with alternative therapies and should be further investigated in this setting.

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Section: Immune Dysregulation Associated With Hlh Results In Signific...mentioning

confidence: 99%

Section: Immune Dysregulation Associated With Hlh Results In Signific...mentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Anakinra versus etoposide‐based therapy added to high‐dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis

Lee,

Cao,

Vittayawacharin

et al. 2023

European J of Haematology

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“…Given the severity of this syndrome with high mortality reports, aggressive treatment is advised. Several studies have shown the efficacy of Anakinra in HLH, especially when it's secondary to rheumatologic disorders [17][18][19]. The patient's treatment included a multimodal approach using the EUROLUPUS protocol and adjunctive therapy for HLH with anakinra.…”

Section: Discussionmentioning

confidence: 99%

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Fernandes¹,

Ferreira²,

Lynce³

et al. 2023

Cureus

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Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect almost every organ. Lupus protein-losing enteropathy (PLE) is one of the rarest manifestations of gastrointestinal involvement. Lupus flare as initial presentation is rare and the disease can act as a trigger to other pathologic immune syndromes like Hemophagocytic Lymphohistiocytosis (HLH), although this association is rare.We report the case of a previously healthy African 39-year-old female patient, with a recent history of cesarean section. Admitted to the Emergency Department (ED) with diffuse abdominal pain and fever, having completed a cycle of antibiotic therapy for initially suspected endometritis. The clinical picture progressed with sustained high fever, new-onset lymphadenopathies, systemic rash, acute pulmonary edema and seizures. Laboratory findings included hyperferritinemia, hypertriglyceridemia, proteinuria and hypoalbuminemia. The auto-immune panel was positive for antinuclear antibodies (ANA), anti-dsDNA, anti-SSA and anti-SSB, anti-PL7, anti-RNP, anti-U1-SnRNP, and anti-Pm-Scl75. She also presented hypocomplementemia. An inaugural flare of SLE with multisystemic involvement and concomitant secondary Hemophagocytic Syndrome was considered and therapy with methylprednisolone pulses, Anakinra and Cyclophosphamide was started. By the end of the first cycle of cyclophosphamide, the patient presented clinical worsening with abdominal pain recrudescence and profuse diarrhea. After the exclusion of an infectious process, a Lupus PLE was assumed and Cyclophosphamide protocol was resumed, with sustained clinical improvement after the induction protocol.Despite initially suspected gynecological infection, the clinical progression with multisystemic involvement together with the auto-immune panel made the diagnosis of SLE possible, with other laboratory findings raising the suspicion of HLH. This case represents a rare report of severe SLE with multiple organ involvement accompanied by HLH. Gastrointestinal involvement with PLE added rarity and morbidity to the clinical picture. The case reinforces the idea that when organ dysfunction is due to a severe autoimmune response, supportive treatment can be lifesaving until immunosuppressive drugs reach their full effect.

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“…Naymagon, for instance, noted in a retrospective study of outcomes associated with anakinra treatment that those with rheumatologic condition-associated MAS experienced 75% OS, versus only 17% survival in patients with other underlying causes of sHLH [ 63 ]. Publications describing the use of anakinra for mHLH have been varied, ranging from frontline monotherapy use in Hodgkin lymphoma to relapsed/refractory HLH in myelodysplastic syndrome (MDS) and T cell lymphoma (TCL) [ 66 , 67 , 68 ]. The use of anti-IL6 therapy for adult HLH (with tocilizumab; no published data using siltuximab) has been extrapolated from its use in cytokine release syndrome (CRS) and COVID-19.…”

Section: Treatment Of Mhlhmentioning

confidence: 99%

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

Lee

Logan

2023

Cancers

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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe, dysregulated inflammation driven by the inability of T cells to clear an antigenic target. When associated with malignancy (mHLH), the HLH syndrome is typically associated with extremely poor survival. Here, we review the diagnosis of secondary HLH (sHLH) syndromes in adults, with emphasis on the appropriate workup and treatment of mHLH. At present, the management of HLH in adults, including most forms of mHLH, is based on the use of corticosteroids and etoposide following the HLH-94 regimen. In some cases, this therapeutic approach may be cohesively incorporated into malignancy-directed therapy, while in other cases, the decision about whether to treat HLH prior to initiating other therapies may be more complicated. Recent studies exploring the efficacy of other agents in HLH, in particular ruxolitinib, offer hope for better outcomes in the management of mHLH. Considerations for the management of lymphoma-associated mHLH, as well as other forms of mHLH and immunotherapy treatment-related HLH, are discussed.

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Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases

Cited by 14 publications

References 13 publications

Anakinra versus etoposide‐based therapy added to high‐dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis

Anakinra versus etoposide‐based therapy added to high‐dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

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