Requirement for etoposide in the initial treatment of Epstein‐Barr virus–associated haemophagocytic lymphohistiocytosis

Abstract

Summary Haemophagocytic lymphohistiocytosis (HLH) is a severe, even fatal, inflammatory condition. Epstein–Barr virus (EBV) infection‐associated HLH (EBV‐HLH) is one of the most common types of secondary HLH. Etoposide is a key drug in HLH‐94/04 regimen. We sought to identify the importance of etoposide in the initial treatment of EBV‐HLH. Ninety‐three patients with EBV‐HLH of all ages admitted to our centre in 2017 were divided into two groups according to whether the initial treatment contained etoposide or not. The survival of the group whose initial therapy included etoposide (Group 1, 52 patients; 6‐month survival rate 0·769) was significantly better than that of the group whose initial therapy did not include etoposide (Group 2, 41 patients; 6‐month survival rate 0·269) (P < 0·001). In patients aged <18 years old, the survival of Group 1 patients was not significantly better than that of Group 2 patients (P = 0·447), in contrast to patients aged ≥18 years, where the survival of Group1 patients was significantly better than that of Group 2 patients (P < 0·001). We concluded that including etoposide in the initial treatment of EBV‐HLH patients can improve their prognosis, especially adult patients. This may be because that adult patients are recognized as “higher risk” patients.

Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria

“…Although primary HLH is associated with genetic defects in the perforin-mediated cytolytic pathway, macrophage activation syndrome (MAS) is considered to be a secondary form of HLH associated with malignancy, infection, and autoimmune diseases [21]. Because viral infections such as EBV are known to cause HLH by triggering uncontrolled lymphoproliferation [22], there is a concern that KFD can be associated with MAS. While MAS is a potentially life-threatening condition that requires early detection, MAS-specific diagnostic criteria have not yet been fully established.…”

Requirement for Containing Etoposide in the Initial Treatment of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

“…The above three reasons lead to LAHS's urgent need for relevant clinical study to play a certain therapeutic guiding signi cance. Etoposide is one of the core drugs in the HLH-94/04 standard protocol, and its importance in the treatment of HLH has been con rmed, such as the improvement of prognosis in the initial treatment of EBV-HLH [7,8,20]. But its signi cance in LAHS treatment has not yet been discussed.…”

“…Etoposide is one of the key drugs in HLH treatment [6], and its importance and necessity, especially in initial treatment of some types of HLH, are getting a lot attention. In previous report, the initial treatment with etoposide is believed to improve the prognosis of EBV-HLH patients, especially in adult patients [7,8]. In the Alison S.…”

Requirement for containing etoposide in the initial treatment of lymphoma associated hemophagocytic lymphohistiocytosis

“…The priority of HLHdirected treatment and lymphoma-directed treatment is still controversial [5,6,13], even though in some clinical observation, many patients died of HLH within 2-4 weeks in spite of continued treatment of the underlying malignancy [6]. Etoposide is one of the core drugs in the HLH-94/2004 standard protocol, and its importance in the treatment and prognosis of HLH has been con rmed repeatedly [8,9,20]. But, its signi cance in LAHS's treatment has not yet been discussed.…”

“…Etoposide is one of the key drugs in HLH treatment [7], and its importance and necessity, especially in initial treatment of some types of HLH, are getting a lot attention. In previous report, the initial treatment with etoposide is believed to improve the prognosis of EBV-HLH patients, especially in adult patients [8,9]. In the Alison S. and Nancy B.…”