Successful management of severe refractory acquired immune bleeding disorder: Prior to insisting surgery

INTRODUCTION

Acquired bleeding disorders are rare and may be missed before surgery. Additionally, they may be refractory to conventional treatments.

PRESENTATION OF CASE

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.

DISCUSSION

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.

CONCLUSION

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.Abbreviations: APTT, activated partial thromboplastin time; VWF Ag, von Willebrand factor antigen; VWF Act, von Willebrand factor activity; IgG, immunoglobulin G; aVWS, acquired von Willebrand syndrome; VWF, von Willebrand factor; MGUS, monoclonal gammopathy of undetermined significance; MMF, mycophenolate mofetil; DIM, dexamethasone, intravenous immunoglobulin, mycophenolate mofetil; VWD, von Willebrand disease