Emapalumab use in malignancy-associated hemophagocytic lymphohistiocytosis in the United States: the REAL-HLH study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод пока не подготовлен
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Zoref-Lorenz Adi, Allen Carl E, Behrens Edward M, Chandrakasan Shanmuganathan, Chien May, Hermiston Michelle L, Hinson Ashley P, Intzes Stefanos, Isakoff Michael S, Leiding Jennifer W, Oladapo Abiola, Panigrahi Arun, Patel Sachit A, Pednekar Priti, Ray Anish K, Rothman Jennifer A, Saldaña Blachy Dávila, Walkovich Kelly J, Weinstein Joanna, Yee John D, Jordan Michael B, Weinstein Joanna, Hinson Ashley P, Allen Carl E, Eckstein Olive S, Gulati Nitya, Chandrakasan Shanmuganathan, Haines Hilary, Nakano Taizo A, Patel Sachit A, Behrens Edward M, Intzes Stefanos, Hays Allyson, Saldaña Blachy Dávila, Jordan Michael B, Zoref-Lorenz Adi, Hanna Rabi, Isakoff Michael, Ray Anish K, Rothman Jennifer A, Tandra Anand, Cooper Robert, Leiding Jennifer W, Chien May, Sarangi Susmita, Gidvani-Diaz Vinod K, Satwani Prakash, Carter John, Henry Michael, Gloude Nicholas, Bhatt Sima, Bhatla Deepika, Draper Lauren, Panigrahi Arun, Hermiston Michelle L, Modica Renee, Riskalla Mona, Baker Ashley, Ward Brant
Аннотация
Malignancy-associated hemophagocytic lymphohistiocytosis (mHLH), a hyperinflammatory syndrome, has poor prognosis and no standard therapy. Emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon gamma, is approved for treating primary hemophagocytic lymphohistiocytosis (pHLH). REAL-HLH, a retrospective chart review conducted across 33 US hospitals, evaluated real-world treatment patterns and outcomes in patients treated with ≥1 dose of emapalumab between 20 November 2018 and 31 October 2021. Data are presented for the subset of patients with mHLH. Overall, 51 of 105 (48.6%) patients were categorized as having secondary HLH, of which 17 of 51 patients had HLH associated with an underlying malignancy (mHLH). At HLH diagnosis, the median age (range) was 15.0 (3.0-27.0) years, 6 of 14 (42.9%) patients with available data had a positive Optimized HLH Inflammatory index, indicating pathologic inflammation; 9 of 17 (52.9%) had infections; and 10 of 17 (58.8%) received emapalumab in an intensive care unit. Emapalumab was primarily initiated for treating refractory (10/17; 58.8%) or progressive (3/17, 17.7%) disease. Most patients received HLH-related therapies before (16/17; 94.1%) and/or concurrent with (15/17; 88.2%) emapalumab. Treatment with emapalumab-containing regimens normalized most key laboratory parameters and maintained normal values for others (fibrinogen [11/13; 84.6%], absolute neutrophil count [6/10; 60%], and chemokine ligand 9 [7/8; 87.5%]) per physician assessment. Overall survival at the end of follow-up and 12-month survival probability from emapalumab initiation were 23.5% and 22.1%, respectively. In conclusion, emapalumab-containing regimens normalized, improved, or maintained normal values for most laboratory parameters in patients with mHLH. Future studies are warranted to establish appropriate emapalumab dosing and utility in this high-risk population.