title: Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria.
abstract: Aberrant smooth muscle dystonia during hemolytic episodes in paroxysmal nocturnal hemoglobinuria (PNH) is implicated in the symptoms of abdominal pain, dysphagia and erectile dysfunction. Here we report two PNH patients treated with the complement inhibitor, eculizumab. Complement inhibition has been sustained for over 2 years and results in resolution of intravascular hemolysis and amelioration of symptoms associated with smooth muscle contractions.
authors.affiliation: Haematology, Leeds Teaching Hospitals NHS Trust, UK. anitahill@nhs.net
authors.forename: Anita
authors.initials: A
authors.lastname: Hill
authors.forename: Russell P
authors.initials: RP
authors.lastname: Rother
authors.forename: Peter
authors.initials: P
authors.lastname: Hillmen
mesh_terms: D015746:Abdominal Pain / Q000188:drug therapy* / Q000209:etiology; D000328:Adult; D000741:Anemia, Aplastic / Q000150:complications; D000911:Antibodies, Monoclonal / Q000494:pharmacology / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D001803:Blood Transfusion; D003182:Complement C5 / Q000037:antagonists & inhibitors; D003680:Deglutition Disorders / Q000188:drug therapy* / Q000209:etiology; D007172:Erectile Dysfunction / Q000188:drug therapy* / Q000209:etiology; D005221:Fatigue / Q000209:etiology; D005260:Female; D006454:Hemoglobins / Q000009:adverse effects; D006457:Hemoglobinuria, Paroxysmal / Q000188:drug therapy* / Q000503:physiopathology; D006461:Hemolysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009119:Muscle Contraction / Q000187:drug effects; D009130:Muscle, Smooth / Q000503:physiopathology*; D009569:Nitric Oxide / Q000502:physiology
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D003182:Complement C5; D006454:Hemoglobins; D009569:Nitric Oxide; C481642:eculizumab