title: Combination treatment with an erythropoiesis-stimulating agent and intravenous iron alleviates anaemia in patients with hereditary haemorrhagic telangiectasia.
abstract: BACKGROUND
  Patients with hereditary haemorrhagic telangiectasia (HHT) suffer from recurrent epistaxis and bleeding from gastrointestinal telangiectasias that occur despite otherwise normal haemostasis and result in iron deficiency anaemia with increasing severity. In advanced disease, anaemia may be severe, be irresponsive to iron supplementation, and may lead to red blood cell transfusion dependency.
  
  
  METHODS
  We conducted a retrospective study at our Centre for Osler's Disease to evaluate the effectiveness of adding an erythropoiesis-stimulating agent (ESA) to intravenous iron supplementation in the management of anaemic HHT patients. Blood values and treatment parameters were collected for nine months before combination therapy (iron supplementation only) and 12 months during combination therapy (iron supplementation plus ESA).
  
  
  RESULTS
  Four patients received intravenous iron and an ESA with mean weekly doses of 126 mg and 17,300 units (U), respectively. Mean haemoglobin improved significantly during combination therapy, from 106 g/L to 119 g/L (p < 0.001).
  
  
  CONCLUSIONS
  Anaemia can be alleviated in patients with HHT who are irresponsive to intravenous iron supplementation, by addition of an ESA. The proposed mechanism behind the iron irresponsiveness is that the anaemia is caused by a combination of recurrent haemorrhage and anaemia of chronic disease.
authors.affiliation: Department of Haematology, Uppsala University Hospital , Uppsala , Sweden.
authors.forename: Honar
authors.initials: H
authors.lastname: Cherif
authors.forename: Torbjörn
authors.initials: T
authors.lastname: Karlsson
mesh_terms: D000368:Aged; D000740:Anemia / Q000188:drug therapy*; D002908:Chronic Disease; D003131:Combined Modality Therapy / Q000379:methods; D005260:Female; D006397:Hematinics / Q000008:administration & dosage*; D006470:Hemorrhage / Q000188:drug therapy / Q000517:prevention & control; D006801:Humans; D007262:Infusions, Intravenous; D007501:Iron / Q000008:administration & dosage* / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000628:therapy*
chemical_list: D006397:Hematinics; D007501:Iron
keywords: Anaemia; erythropoiesis-stimulating agents; hereditary haemorrhagic telangiectasia; iron