title: Short-term oral corticosteroid therapy for acute haemarthrosis in haemophilia patients with high-titre inhibitors.
abstract: Treatment of acute bleeding events is unsatisfactory in patients with haemophilia and high-titre factor VIII (FVIII) inhibitors. In order to determine whether short-term corticosteroid therapy enhances resolution of the signs and symptoms of acute haemarthrosis, we performed a randomized, double-blind, placebo-controlled study in children with FVIII deficiency and high-titre inhibitors receiving Factor Eight Inhibitor Bypass Activity (FEIBA) for acute haemorrhagic events. At each haemarthrosis, patients were randomized to receive either prednisolone 2 mg kg(-1) day(-1) or placebo-divided t.i.d. for 2 days (six doses) in addition to FEIBA. The primary endpoint was the number of subsequent doses of FEIBA required. The effect of the study medication was also assessed subjectively by patients or parents, by physical examination and by repeated haemorrhages into the joint. During the study period, seven patients were enrolled with 45 evaluable events, 24 treated with prednisolone and 21 with placebo. An average of 2.08 and 1.86 doses of FEIBA were infused in the prednisolone- and placebo-treated patients, respectively. By Wilcoxon Rank Sum Test, there was no statistically significant difference in number of additional infusions of FEIBA or duration of symptoms between the corticosteroid and placebo arms. We conclude that there is no significant benefit of a 2-day course of oral corticosteroids as adjunctive therapy for haemarthrosis in patients with haemophilia and a high-titre inhibitor.
authors.affiliation: The University of Texas Southwestern Medical Center at Dallas, Children's Medical Center Dallas, Division of Hematology-Oncology Department of Pediatrics, Dallas, Texas, USA. desireem@kapiolani.org
authors.forename: D
authors.initials: D
authors.lastname: Medeiros
authors.forename: J A
authors.initials: JA
authors.lastname: Laufenberg
authors.forename: K L
authors.initials: KL
authors.lastname: Miller
authors.forename: G R
authors.initials: GR
authors.lastname: Buchanan
mesh_terms: D000293:Adolescent; D000328:Adult; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003029:Coagulants / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use*; D006395:Hemarthrosis / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000188:drug therapy; D006801:Humans; D010146:Pain / Q000188:drug therapy / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use*; D018709:Statistics, Nonparametric; D017211:Treatment Failure
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D003029:Coagulants; D005938:Glucocorticoids; D011239:Prednisolone; C065655:anti-inhibitor coagulant complex