Chimeric antigen receptor T-cell therapy associated hemophagocytic lymphohistiocytosis syndrome: clinical presentation, outcomes, and management
Arushi Khurana, Allison Rosenthal, Razan Mohty, Mamatha Gaddam, Radhika Bansal, Matthew Hathcock, Adrienne Nedved, Urshila Durani, Madiha Iqbal, Yucai Wang, Jonas Paludo, José C. Villasboas, David Dingli, Taxiarchis Kourelis, Nelson Leung, Hassan B. Alkhateeb, Michael W. Ruff, Alice Gallo de Moraes, Paschalis Vergidis, Joerg Herrmann, Saad S. Kenderian, N. Nora Bennani, Patrick B. Johnston, Stephen M. Ansell, Yi Lin
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of chimeric antigen receptor T-cell (CAR-T) therapy [ 1 , 2 , 3 ]. With the increasing use of CAR-T therapy in treating hematological malignancies, the unique phenomenon of CAR-T-associated HLH (carHLH) is increasingly recognized. Albeit rare, reported rates vary from 1–3% to 35% depending on the underlying disease, patient population and the CAR-T product [ 1 , 2 , 3 ]. Recently ASTCT has published guidelines recognizing HLH in the setting of CAR-T as immune effector cell-associated HLH-like syndrome (IEC-HS) [ 4 ]. The most extensive series to date that comprehensively described manifestations, along with patient and CAR-T specific factors associated with the development of carHLH, is that of anti-CD22 CAR-T in 21 pediatric patients with B-ALL [ 2 ]. We herein provide a detailed description of clinical presentation, treatment strategies, and outcomes in adult patients who developed carHLH across three sites at Mayo Clinic.