title: Ruxolitinib treatment for GvHD in patients with myelofibrosis.
abstract: Jak1/2 inhibitor ruxolitinib is a promising agent for treating steroid-refractory GvHD after allogeneic hematopoietic stem cell transplantation (SCT) to produce quick and durable responses. However, optimal dose and tapering schedule of ruxolitinib remain to be determined. Discontinuation of ruxolitinib in myelofibrosis often induces 'withdrawal syndrome' characterized by acute relapse of the disease, but this issue is not well addressed in the treatment of GvHD. Four patients with GvHD (one acute and three chronic) after SCT for myelofibrosis were treated with ruxolitinib. Low-dose ruxolitinib at 5 mg/day was safe and effective, but one of two patients treated at 10 mg/day of ruxolitinib was complicated with severe cytopenia. Withdrawal syndrome developed in one patient, who died of recurrence of GvHD shortly after discontinuation of ruxolitinib. Slow tapering or maintenance with low-dose ruxolitinib inhibited GvHD flare. Our experience calls attention that initiation at low-dose of ruxolitinib may be safe and careful tapering schedule is required to avoid withdrawal syndrome in patients with GvHD after SCT for myelofibrosis.
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Mori
authors.affiliation: Department of Cardiology and Hematology, Fukushima, Japan.|Department of Blood Transfusion and Transplantation Immunology, Fukushima Medical University, Fukushima, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Ikeda
authors.affiliation: Department of Hematology and Oncology, Okayama University Hospital, Okayama, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Inomata
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
authors.forename: G
authors.initials: G
authors.lastname: Yoshimoto
authors.affiliation: Department of Hematology and Oncology, Okayama University Hospital, Okayama, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Fujii
authors.affiliation: Division of Hematology and Oncology, Shimane Prefectural Central Hospital, Izumo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Ago
authors.affiliation: Department of Hematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Teshima
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006086:Graft vs Host Disease / Q000188:drug therapy*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006801:Humans; D053612:Janus Kinases / Q000037:antagonists & inhibitors; D008875:Middle Aged; D009570:Nitriles; D055728:Primary Myelofibrosis / Q000150:complications / Q000628:therapy*; D011720:Pyrazoles / Q000008:administration & dosage* / Q000009:adverse effects; D011743:Pyrimidines; D013375:Substance Withdrawal Syndrome / Q000517:prevention & control; D016896:Treatment Outcome
chemical_list: D009570:Nitriles; D011720:Pyrazoles; D011743:Pyrimidines; C540383:ruxolitinib; D053612:Janus Kinases