title: Selective splenic artery embolization for the treatment of thrombocytopenia and hypersplenism in paroxysmal nocturnal hemoglobinuria.
abstract: BACKGROUND
  PNH is associated with abdominal vein thrombosis, which can cause splenomegaly and hypersplenism. The combination of thrombosis, splenomegaly, and thrombocytopenia (TST) is challenging because anticoagulants are indicated but thrombocytopenia may increase the bleeding risk. Splenectomy could alleviate thrombocytopenia and reduce portal pressure, but it can cause post-operative thromboses and opportunistic infections. We therefore sought to determine whether selective splenic artery embolization (SSAE) is a safe and effective alternative to splenectomy for TST in patients with PNH.
  
  
  METHODS
  Four patients with PNH and TST received successive rounds of SSAE. By targeting distal vessels for occlusion, we aimed to infarct approximately 1/3 of the spleen with each procedure.
  
  
  RESULTS
  Three of 4 patients had an improvement in their platelet count, and 3 of 3 had major improvement in abdominal pain/discomfort. The one patient whose platelet count did not respond had developed marrow failure, and she did well with an allo-SCT. Post-procedure pain and fever were common and manageable; only one patient developed a loculated pleural effusion requiring drainage. One patient, who had had only a partial response to eculizumab, responded to SSAE not only with an improved platelet count, but also with an increase in hemoglobin level and decreased transfusion requirement.
  
  
  CONCLUSIONS
  These data indicate that SSAE can decrease spleen size and reverse hypersplenism, without exposing the patient to the complications of splenectomy. In addition, SSAE probably reduces the uptake of opsonised red cells in patients who have had a limited response to eculizumab, resulting in an improved quality of life for selected patients.
authors.affiliation: Division of Hematology, NYU School of Medicine, and the New York Harbor VA Medical Center, New York, NY, USA. david.araten@nyumc.org.
authors.forename: David J
authors.initials: DJ
authors.lastname: Araten
authors.forename: Anna Paola
authors.initials: AP
authors.lastname: Iori
authors.forename: Karen
authors.initials: K
authors.lastname: Brown
authors.forename: Giovanni Fernando
authors.initials: GF
authors.lastname: Torelli
authors.forename: Walter
authors.initials: W
authors.lastname: Barberi
authors.forename: Fiammetta
authors.initials: F
authors.lastname: Natalino
authors.forename: Maria Stefania
authors.initials: MS
authors.lastname: De Propris
authors.forename: Corrado
authors.initials: C
authors.lastname: Girmenia
authors.forename: Filippo Maria
authors.initials: FM
authors.lastname: Salvatori
authors.forename: Orly
authors.initials: O
authors.lastname: Zelig
authors.forename: Robin
authors.initials: R
authors.lastname: Foà
authors.forename: Lucio
authors.initials: L
authors.lastname: Luzzatto
mesh_terms: D000293:Adolescent; D000328:Adult; D004621:Embolization, Therapeutic / Q000379:methods*; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000628:therapy*; D006801:Humans; D006971:Hypersplenism / Q000628:therapy*; D008297:Male; D013157:Splenic Artery; D013921:Thrombocytopenia / Q000628:therapy*; D055815:Young Adult