Haematologica
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Haematologica, Vol 90, Issue 12_Suppl, ECR40-ECR40
Copyright © 2005 by Ferrata Storti Foundation

Case Reports

Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria

A Hill, RP Rother, and P Hillmen

Haematology, Leeds Teaching Hospitals NHS Trust, UK. anitahill@nhs.net

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.




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Copyright © 2005 by the Ferrata Storti Foundation.