Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria.
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.
Hill
Anita
A
Haematology, Leeds Teaching Hospitals NHS Trust, UK. anitahill@nhs.net
Rother
Russell P
RP
Hillmen
Peter
P
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
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D003182:Complement C5; D006454:Hemoglobins; D009569:Nitric Oxide; C481642:eculizumab