Ruxolitinib for hematopoietic cell transplantation-associated hemophagocytic lymphohistiocytosis
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CASE REPORT
Ruxolitinib for hematopoietic cell transplantation‑associated hemophagocytic lymphohistiocytosis Rintaro Ono1 · Miho Ashiarai1 · Shinsuke Hirabayashi2 · Kazuyoshi Mizuki1 · Yosuke Hosoya1 · Hiroki Yoshihara1 · Junya Ohtake3 · Shinichiro Mori4 · Atsushi Manabe2 · Daisuke Hasegawa1 Received: 30 June 2020 / Revised: 7 August 2020 / Accepted: 1 September 2020 © Japanese Society of Hematology 2020
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a severe complication after allogeneic hematopoietic cell transplantation (HCT) and can cause graft failure or multi-organ failure. Here, we report two children with refractory HCT-associated HLH treated with ruxolitinib. In the first patient, ruxolitinib resolved fever, cytopenia and hyperferritinemia. In another patient, although severe hepatic failure, which developed and worsened before the administration of ruxolitinib, was irreversible, rapid improvement in fever, leukopenia and hyperferritinemia was observed. Of note, multiplex cytokine profiling showed amelioration of cytokine storm in both patients. Ruxolitinib may be an encouraging option for HCT-associated HLH. Keywords Ruxolitinib · Hemophagocytic lymphohistiocytosis · Hematopoietic cell transplantation · Cytokine profiling
Introduction Hemophagocytic lymphohistiocytosis (HLH) is characterized by overactivation of T cells and macrophages and, consequently, cytokine storm. It is classified as primary or secondary, which is associated with infections, autoimmune diseases, and malignancies. HLH also occurs after allogeneic hematopoietic cell transplantation (HCT) and can cause graft failure and fatal multi-organ failure [1]. First-line treatment for HLH has been standardized as immunochemotherapy consisting of dexamethasone, etoposide, and cyclosporine. However, HCT-associated HLH is often treated with corticosteroids, intravenous immunoglobulin (IVIG), and low-dose etoposide to avoid the possible Rintaro Ono and Miho Ashiarai contributed equally to this work. * Daisuke Hasegawa [email protected] 1
Department of Pediatrics, St. Luke’s International Hospital, 9‑1 Akashicho, Chuo‑ku, Tokyo 104‑0044, Japan
2
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
3
Center for Medical Sciences, St Luke’s International University, Tokyo, Japan
4
Department of Hematology, St. Luke’s International Hospital, Tokyo, Japan
excessive toxicities. Recently, cytokine-targeted therapies have been developed, although their efficacy has not been proven [2]. Ruxolitinib is a JAK1/2 inhibitor that inhibits multiple cytokine signaling and has shown efficacy in both preclinical models [3, 4] and clinical cases of HLH [5–11]. Herein, we report two children with HCT-associated HLH treated with ruxolitinib.
Case reports Patient 1 A 16-month-old female was diagnosed with B-cell precursor acute lymphoblastic leukemia without known cytogenetic abnormalities. Although molecular remission was achieved after induction chemotherapy, she suffered from isolated bone marrow (BM
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