Contributions
Abstract: PB1810
Type: Publication Only
Background
Acquired hemophilia (AH) is a rare autoimmune bleeding disorder caused by the development of an autoantibody directed against a coagulation factor, most frequently factor VIII (FVIII).
Aims
To review the characteristics and management of AH in our center during the last five years (2013-2017).
Methods
Retrospective single-center study. There is no Department of Obstetrics in our hospital. Data was collected from clinical records.
Results
Eleven patients were found: 10 with FVIII inhibitor and 1 with FXI inhibitor.
Of the 10 patients diagnosed with AH A (4M, 6F), 1 was a relapse. The median age at diagnosis was 85 years (76-93). Median time to diagnosis from symptom onset was 1 month (0.4-18). Underlying disease: autoimmune (4), solid neoplasia (3), hematologic neoplasia (2) and idiopathic (1). All cases had bleeding as initial presentation (one life-threatening): subcutaneous (6), genitourinary (3), muscle (1), gastrointestinal (1) and/or after surgery (1). All patients had anaemia at diagnosis. FVIII level at diagnosis was <5% in most cases (8/10), the median inhibitor level was 64 BU (1.4-134). Regarding treatment, 3 patients received Corticosteroids alone due to comorbidities, without follow-up; 3/3 died due to underlying disease/unknown cause. The remaining 7 patients were treated to eradicate FVIII inhibitor (Table 1). Recombinant FVII activated was chosen as first-line haemostatic agent. Bleeding stopped in all cases and none presented thrombosis. Three of these 7 patients passed away, 2 due to underlying disease/unknown cause and 1 due to immunosuppression.
The patient with acquired FXI inhibitor was a 73 year-old female with no underlying disease. No bleeding at diagnosis. The first-line of eradicator treatment was CS + CTX, then second-line Rituximab, without response. She passed away due to immunosuppression-related complications.
Table 1. Summary of the 7 patients who received treatment to eradicate FVIII inhibitor | ||||||||
Underlying disease (age/sex) | FVIII (%) | Inhibitor (BU) | 1st line treatment | 2nd line treatment | Time to response*** (days) | Complications | Relapse *** | |
Partial | Complete | |||||||
Neoplasia (85/M) | 5 | NA | CS + CTX | - | 49 | - | - | Yes |
Idiopathic (81/F) | <5 | NA | CS + CTX | - | 51 | 61 | - | - |
Autoimmune (88/F)* | <5 | 32 | CS + CTX | - | 30 | 60 | - | Yes |
Autoimmune (85/M) | 7 | 1.4 | CS + Rituximab | - | 17 | 48 | Sepsis | - |
Neoplasia (77/M) | <5 | 95 | MBMP** | - | 24 | - | IFI, CMV | - |
Autoimmune (76/F) | <5 | 64 | MBMP | Rituximab | 20 | 55 | - | - |
Neoplasia (79/F) | <5 | 64 | MBMP | Rituximab | 27 | 51 | Neutropenia | - |
*Relapse; **no plasmapheresis; ***GTH-AH 01/2010. CMV: Cytomegalovirus; CS: Corticosteroids; CTX: Cyclophosphamide; IFI: Invasive Fungal Infection; MBMP: Modified Bonn-Malmö Protocol; NA: Not Available.
Conclusion
All patients were elderly at diagnosis, influenced by the lack of pregnancy-associated cases. An underlying disease was found in almost all cases. In our experience all patients with FVIII inhibitor who received eradicator treatment achieved a response (partial +/- complete). Treatment with higher immunosuppression involved more complications. Most deaths were due to underlying disease/unknown cause.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired hemophilia, Bleeding, Factor VIII Inhibitor, Immunosuppressive therapy
Abstract: PB1810
Type: Publication Only
Background
Acquired hemophilia (AH) is a rare autoimmune bleeding disorder caused by the development of an autoantibody directed against a coagulation factor, most frequently factor VIII (FVIII).
Aims
To review the characteristics and management of AH in our center during the last five years (2013-2017).
Methods
Retrospective single-center study. There is no Department of Obstetrics in our hospital. Data was collected from clinical records.
Results
Eleven patients were found: 10 with FVIII inhibitor and 1 with FXI inhibitor.
Of the 10 patients diagnosed with AH A (4M, 6F), 1 was a relapse. The median age at diagnosis was 85 years (76-93). Median time to diagnosis from symptom onset was 1 month (0.4-18). Underlying disease: autoimmune (4), solid neoplasia (3), hematologic neoplasia (2) and idiopathic (1). All cases had bleeding as initial presentation (one life-threatening): subcutaneous (6), genitourinary (3), muscle (1), gastrointestinal (1) and/or after surgery (1). All patients had anaemia at diagnosis. FVIII level at diagnosis was <5% in most cases (8/10), the median inhibitor level was 64 BU (1.4-134). Regarding treatment, 3 patients received Corticosteroids alone due to comorbidities, without follow-up; 3/3 died due to underlying disease/unknown cause. The remaining 7 patients were treated to eradicate FVIII inhibitor (Table 1). Recombinant FVII activated was chosen as first-line haemostatic agent. Bleeding stopped in all cases and none presented thrombosis. Three of these 7 patients passed away, 2 due to underlying disease/unknown cause and 1 due to immunosuppression.
The patient with acquired FXI inhibitor was a 73 year-old female with no underlying disease. No bleeding at diagnosis. The first-line of eradicator treatment was CS + CTX, then second-line Rituximab, without response. She passed away due to immunosuppression-related complications.
Table 1. Summary of the 7 patients who received treatment to eradicate FVIII inhibitor | ||||||||
Underlying disease (age/sex) | FVIII (%) | Inhibitor (BU) | 1st line treatment | 2nd line treatment | Time to response*** (days) | Complications | Relapse *** | |
Partial | Complete | |||||||
Neoplasia (85/M) | 5 | NA | CS + CTX | - | 49 | - | - | Yes |
Idiopathic (81/F) | <5 | NA | CS + CTX | - | 51 | 61 | - | - |
Autoimmune (88/F)* | <5 | 32 | CS + CTX | - | 30 | 60 | - | Yes |
Autoimmune (85/M) | 7 | 1.4 | CS + Rituximab | - | 17 | 48 | Sepsis | - |
Neoplasia (77/M) | <5 | 95 | MBMP** | - | 24 | - | IFI, CMV | - |
Autoimmune (76/F) | <5 | 64 | MBMP | Rituximab | 20 | 55 | - | - |
Neoplasia (79/F) | <5 | 64 | MBMP | Rituximab | 27 | 51 | Neutropenia | - |
*Relapse; **no plasmapheresis; ***GTH-AH 01/2010. CMV: Cytomegalovirus; CS: Corticosteroids; CTX: Cyclophosphamide; IFI: Invasive Fungal Infection; MBMP: Modified Bonn-Malmö Protocol; NA: Not Available.
Conclusion
All patients were elderly at diagnosis, influenced by the lack of pregnancy-associated cases. An underlying disease was found in almost all cases. In our experience all patients with FVIII inhibitor who received eradicator treatment achieved a response (partial +/- complete). Treatment with higher immunosuppression involved more complications. Most deaths were due to underlying disease/unknown cause.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired hemophilia, Bleeding, Factor VIII Inhibitor, Immunosuppressive therapy