title: Deviating from safety guidelines during deferiprone therapy in clinical practice may not be associated with higher risk of agranulocytosis.
abstract: BACKGROUND
  A risk associated with the iron chelator deferiprone is the development of neutropenia or agranulocytosis. Accordingly, the product label recommends weekly blood monitoring and immediate interruption of treatment upon detection of an absolute neutrophil count (ANC) <1.5 × 10(9)/L, out of concern that continued therapy might lead to a more severe drop. However, it is uncertain how these recommendations are followed under real-life conditions and, if they are not followed, whether continuation of therapy results in increased incidence of agranulocytosis.
  
  
  METHODS
  This non-interventional surveillance program assessed the monitoring of deferiprone therapy in clinical practice. A total of 294 patients with transfusion-dependent anemias received deferiprone, as monotherapy or with another chelator, for up to 1 year. The participating physicians were not given any instructions about treatment and monitoring beyond being referred to the information in the package insert.
  
  
  RESULTS
  ANC monitoring was conducted at an average interval of 5 ± 4 weeks, and deferiprone was not always interrupted upon detection of neutropenia. One patient (0.3%) experienced agranulocytosis, and nine others (3%) experienced a total of 11 episodes of neutropenia. All neutropenia episodes resolved; median time to resolution was similar whether or not treatment was interrupted; and no case of neutropenia progressed to agranulocytosis.
  
  
  CONCLUSIONS
  These data indicate that less frequent ANC monitoring and continuation of deferiprone therapy during neutropenia are not associated with prolonged neutropenia or with progression to agranulocytosis.
authors.affiliation: Ain Shams University, Cairo, Egypt.
authors.forename: Mohssen
authors.initials: M
authors.lastname: Elalfy
authors.forename: Yasser A
authors.initials: YA
authors.lastname: Wali
authors.forename: Mohamad
authors.initials: M
authors.lastname: Qari
authors.forename: Ghazi
authors.initials: G
authors.lastname: Al Damanhouri
authors.forename: Youssef
authors.initials: Y
authors.lastname: Al-Tonbary
authors.forename: Dilek
authors.initials: D
authors.lastname: Yazman
authors.forename: Zakaria
authors.initials: Z
authors.lastname: Al Hawsawi
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Karakas
authors.forename: Yurdanur
authors.initials: Y
authors.lastname: Kilinc
authors.forename: M Akif
authors.initials: MA
authors.lastname: Yesilipek
authors.forename: Mohamed
authors.initials: M
authors.lastname: Badr
authors.forename: Usama
authors.initials: U
authors.lastname: Elsafy
authors.forename: Mostafa
authors.initials: M
authors.lastname: Salama
authors.forename: Yousryeia
authors.initials: Y
authors.lastname: Abdel Rahman
authors.forename: Shebl
authors.initials: S
authors.lastname: Shebl
authors.forename: Anne
authors.initials: A
authors.lastname: Stilman
authors.forename: Noemi
authors.initials: N
authors.lastname: Toiber Temin
authors.forename: Fernando
authors.initials: F
authors.lastname: Tricta
mesh_terms: D000293:Adolescent; D000328:Adult; D000380:Agranulocytosis / Q000139:chemically induced / Q000517:prevention & control*; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D000077543:Deferiprone; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D019190:Iron Overload / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000139:chemically induced / Q000517:prevention & control*; D009504:Neutrophils; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians'; D011446:Prospective Studies; D011728:Pyridones / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone
keywords: agranulocytosis; deferiprone; hemoglobinopathies; neutropenia; red blood cell disorders; thalassemia