Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) following treatment with tisagenlecleucel

Abstract

Chimeric antigen receptor (CAR) T cell–related HLH/MAS is an unusual manifestation of severe cytokine release syndrome (CRS) with poor prognosis and a challenging diagnosis. The establishment of specific diagnosis criteria is essential, and the combination of several techniques for CAR T‐cell follow‐up, allows a more precise management of this complication.

Dramatic Recovery after Etoposide Phosphate Infusion for Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome following Treatment with Tisagenlecleucel in a Young Patient with Relapsed Acute Lymphoblastic Leukemia: A Case Report

“…Few cases of post-anti-CD19 CAR T-cell therapy for HLH/MAS have been reported so far, and the best treatment for such a complication has not been established [15]. Here, the ultimate use of EP after failure of tocilizumab and siltuximab resulted in a dramatic and unexpected clinical recovery.…”

“…Recently, the cases of 2 patients with suspected HLH/MAS following treatment with tisagenlecleucel have been reported, one treated with cyclophosphamide and extracorporeal cytokine adsorption and the other one with dexamethasone, EP, and anakinra, a recombinant IL-1 receptor antagonist. Both these patients however soon died in progression, even if the second one showed some HLH/MAS improvement [18]. Another case of response to anakinra has been reported [19].…”

Anakinra to Mitigate Hemophagocytic Lymphohistiocytosis-Like Toxicity Following Chimeric Antigen Receptor T-cell Therapy in Pediatric B-cell ALL

“…These studies suggested that anakinra might serve as a steroid-sparing agent and usually was the last medication to be weaned without impacting CAR T-cell efficacy [12,14]. Given the proposed clinical benefits of using anakinra, the prospective trials for preemptive use of anakinra for patients who are at high risk of developing severe CRS and/or carHLH are urgently needed.…”

Advances and perspective of the immunotherapy for relapsed/refractory multiple myeloma (interview)

“…Симпто мы могут варьировать от едва заметных до угрожающих жизни гипотензии и дыхательной недостаточности. В крайней форме системный воспалительный ответ реализуется как фульминантный гемофагоцитарный лимфогистиоцитоз / синдром активации макрофагов (HLH / MAS), ассоциирующийся с очень высокой смертностью [39]. Существует очевидная взаимосвязь между объемом опухолевой массы и тя жестью СВЦ.…”