Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases

被引：15

作者：

Baverez, Clara ^{[1
]}

Grall, Maximilien ^{[2
]}

Gerfaud-Valentin, Mathieu ^{[1
]}

De Gail, Sarah ^{[3
]}

Belot, Alexandre ^{[4
,5
]}

Perpoint, Thomas ^{[6
]}

Weber, Emmanuelle ^{[1
]}

Reynaud, Quitterie ^{[7
,8
]}

Seve, Pascal ^{[1
,8
]}

Jamilloux, Yvan ^{[1
,5
]}

机构：

[1] Univ Claude Bernard Lyon 1, Hosp Civils Lyon, Serv Med Interne, Hop Croix Rousse, F-69008 Lyon, France

[2] Univ Rouen, CHU Rouen, Serv Med Intens & Reanimat, F-76000 Rouen, France

[3] Univ Rouen, Hop Hospitalo Univ Rouen, Serv Med Interne, F-76000 Rouen, France

[4] Univ Claude Bernard Lyon 1, Hosp Civils Lyon, Serv Rhumato Nephro Dermatol Pediat, Hop Femme Mere Enfants, F-69008 Lyon, France

[5] Univ Claude Bernard Lyon 1, Lyon Immunopathol FEderat LIFE, F-69100 Lyon, France

[6] Univ Claude Bernard Lyon 1, Hosp Civils Lyon, Serv Malad Infect & Trop, Hop Croix Rousse, F-69008 Lyon, France

[7] Univ Claude Bernard Lyon 1, Hosp Civils Lyon, Ctr Hosp Lyon Sud, Serv Med Interne, F-69008 Lyon, France

[8] Univ Claude Bernard Lyon 1, INSERM U1290, Res Healthcare Performance RESHAPE, F-69100 Lyon, France

来源：

JOURNAL OF CLINICAL MEDICINE | 2022年 / 11卷 / 19期

关键词：

anakinra; interleukin-1; hemophagocytic lymphohistiocytosis; adult onset Still's disease; immunotherapy; cytokines; inflammatory mediators; MACROPHAGE ACTIVATION SYNDROME; DIAGNOSIS; ADULTS;

D O I：

10.3390/jcm11195799

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening cytokine storm syndrome. There are no definitive guidelines for the management of secondary HLH (sHLH). A recent report by a National Health Service (NHS) clinical panel has recommended anakinra as a treatment option. We aimed to analyse the efficacy and safety of anakinra for the treatment of all-cause sHLH. We conducted a multicentric retrospective study in two French University hospitals and included all patients who had a diagnosis of sHLH and who received anakinra. Among 21 patients (median age, 45 years), 13 were men. Anakinra was used as first-line therapy in 10 patients, and as monotherapy in 5 patients. We found that anakinra was effective in 19/21 patients (90.5%), with fever resolution in 19 patients (90.5%) within a median of 1.0 day (1, 2). At the Day 7 assessment, the mean CRP concentration decreased significantly (p < 0.001), as did the mean ferritin (p = 0.011). Anakinra was generally safe and well tolerated and was discontinued for side effects in only three patients (14.3%). Anakinra is an efficient and safe treatment to control sHLH of various causes. These data, together with the recent report of the NHS panel, call for the rapid conduct of prospective randomized clinical trials.

引用

页数：9

共 14 条

[1] Haemophagocytic lymphohistiocytosis in adult critical care [J].