title: Systemic Therapy In Acquired Haemophilia - A Single Institute Experience.
abstract: A cornerstone of the management of Acquired Haemophilia A (AHA) involves inhibitor eradication. First line immunosuppressive agents are usually steroids, either alone or in combination with cyclophosphamide. We present the use of Rituximab, cyclophosphamide, vincristine and prednisolone (RCVP) combination as immunosuppressant in AHA in a small cohort of patients in order to control their symptoms and eradicate inhibitors. This was a retrospective analysis of all AHA patients treated at the Northern Ireland Haemophilia centre over a six year period. During this time, a total of six patients were newly diagnosed with AHA. Four of these patients failed to respond conventional therapy of steroids and cyclophosphamide, they were however successfully treated with RCVP/ RCV. All patients achieved complete remission with this regimen after 1 to 2 cycles of treatment. Remission has been maintained for an extended time period (range 33-69 months). As AHA is related to immune modulation and, in some cases, underlying malignancy we decided to use this regime as it is effective in either condition. From our experience, we demonstrate that RCVP combination is a promising treatment in patients with AHA who fail to respond to steroids alone or who have been on pre-existing immunosuppression.
authors.affiliation: Northern Ireland Regional haemophilia Centre, Belfast City Hospital, Belfast, Northern Ireland.
authors.forename: Lawless
authors.initials: L
authors.lastname: Sarah
authors.affiliation: Northern Ireland Cancer Centre, Belfast City Hospital, Belfast, Northern Ireland.
authors.forename: Das
authors.initials: D
authors.lastname: Prantik
authors.affiliation: Northern Ireland Regional haemophilia Centre, Belfast City Hospital, Belfast, Northern Ireland.
authors.forename: Benson
authors.initials: B
authors.lastname: Gary
mesh_terms: D000368:Aged; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D000069283:Rituximab / Q000627:therapeutic use*; D014750:Vincristine / Q000627:therapeutic use*
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D014750:Vincristine; D003520:Cyclophosphamide
keywords: RCVP; acquired haemophilia; inhibitor eradication