title: Successful management of severe refractory acquired immune bleeding disorder: Prior to insisting surgery.
abstract: BACKGROUND
  Acquired bleeding disorders are rare and may be missed before surgery. Additionally, they may be refractory to conventional treatments.
  
  
  METHODS
  A 50-year-old patient experienced prolonged post-operative bleeding when his bleeding disorder was missed prior to his undergoing inguinal herniorrhaphy. Post-operative investigations revealed severe acquired von Willebrand syndrome associated with a monoclonal gammopathy of undetermined significance. A few months later, he required umbilical herniorrhaphy, but he did not respond to attempts to raise his von Willebrand factor antigen and activity levels using conventional therapies, including desmopressin, cryoprecipitate, intravenous immunoglobulin, and Von Willebrand factor concentrate. A triple therapy combination of dexamethasone, intravenous immunoglobulin, and mycophenolate mofetil was administered, with a successful and sustained response, lasting about 2 months. The surgery was performed safely, without any complications.
  
  
  CONCLUSIONS
  Conventional acquired von Willebrand syndrome treatment is usually aimed at replacing von Willebrand factor or stimulating its secretion from storage in endothelial cells. In the present case, the alternative treatment was directed against both the humoral and cell-mediated immune mechanisms.
  
  
  CONCLUSIONS
  This case of acquired bleeding disorder showed that more attention must be given to a patient's coagulation profile, even if only very minor laboratory coagulation derangements are detected prior to surgery, to avoid missing such rare disorders. The described triple therapy demonstrated good effects and may be considered for inclusion in a controlled randomized study to determine its usefulness for other surgeries delayed due to severe acquired bleeding disorders. To the best of our knowledge, this triple combination treatment has not been previously used for the treatment of severe acquired bleeding disorders that are refractory to conventional therapies.
authors.affiliation: Department of Hematology, Amiri Hospital, Kuwait City, Kuwait. Electronic address: Cbc9@hotmail.com.
authors.forename: Hassan
authors.initials: H
authors.lastname: Al-Jafar
authors.affiliation: Department of Surgery, Amiri Hospital, Kuwait City, Kuwait. Electronic address: Dralbarjas@yahoo.com.
authors.forename: H
authors.initials: H
authors.lastname: Al-Barjas
authors.affiliation: Department of Emergency Medicine and Trauma, Amiri Hospital, Kuwait City, Kuwait. Electronic address: docraed@gmail.com.
authors.forename: Raed A
authors.initials: RA
authors.lastname: Hashem
authors.affiliation: Department of Clinical Chemistry, Amiri Hospital, Kuwait City, Kuwait. Electronic address: Kotb.tha@gmail.com.
authors.forename: Thanaa M K
authors.initials: TM
authors.lastname: Refaii
authors.affiliation: Department of Pharmacy, Amiri Hospital, Kuwait City, Kuwait. Electronic address: Pharma22@hotmail.com.
authors.forename: Ahmad M
authors.initials: AM
authors.lastname: AlSaeed
keywords: Acquired bleeding disorder; Acquired von Willebrand syndrome; Dexamethasone; Intravenous immunoglobulin; Monoclonal gammopathy; Mycophenolate mofetil; Paraprotein; Surgery