ACQUIRED HAEMOPHILIA: A MALAYSIAN OUTLOOK
(Abstract release date: 05/19/16)
EHA Library. Selvaratnam V. 06/09/16; 132541; E992
Dr. Veena Selvaratnam
Contributions
Contributions
Abstract
Abstract: E992
Type: Eposter Presentation
Background
Acquired Factor VIII inhibitor is a very rare bleeding condition due to the spontaneous development of autoantibodies directed against factor VIII.
Aims
To identify at the demographics, presenting features and bleeding characteristics of patients with Acquired Hemophilia referred to the Haematology Unit at Ampang General Hospital, over a 10 year period.
Methods
The list of patients with acquired factor VIII inhibitor who were treated from June 2005 to May 2015 was identified and their case notes were then retrieved from the medical records of the hospital.
Results
Between 2005 and 2015, 28 patients from Kuala Lumpur were diagnosed with acquired factor VIII inhibitors. The population of Kuala Lumpur is 1,501,800 (Population Statistics 2003) giving an incidence of 1.8 cases /million/year.The patient demographics are shown in Table 1.Ten patients (36%) had an underlying associated disease. One was postpartum with history of psoriasis, two had systemic lupus erythematosus, three with bullous pemphigoid, two with thyroid disease, one rheumatoid arthritis and one with a malignant lung nodule. In the remaining 18 patients (64%) were idiopathic. All patients had abnormal bleeding with a total of 54 haemorrhagic episodes. 14 patients had life or limb threatening bleeding: postpartum haemorrhage, iliopsoas haematoma, compartment syndrome, arterial puncture, gastrointestinal bleed and intracranial bleed. Table 1. Demographics of patients with acquired FVIII inhibitor,
Table 2. Sites of Haemorrhages on Presentation
Conclusion
Over a 10-year period we treated 28 patients with acquired hemophilia with an estimated incidence of 1.8/million/year. The median age of was 58.5 years, with a male preponderance(57%). It was associated with an underlying disease in 36% of cases. Life or limb threatening haemorrhages were seen in 50% of patients. Factor VIII level and inhibitor titre at presentation did not predict severity of bleeding.Mortality rate was 15%; all patients died of complications related to bleeding and treatment. It is important to recognize and diagnose this disorder, as appropriate treatment will give a favorable outcome; failing which it can be fatal.
Session topic: E-poster
Keyword(s): Acquired hemophilia
Type: Eposter Presentation
Background
Acquired Factor VIII inhibitor is a very rare bleeding condition due to the spontaneous development of autoantibodies directed against factor VIII.
Aims
To identify at the demographics, presenting features and bleeding characteristics of patients with Acquired Hemophilia referred to the Haematology Unit at Ampang General Hospital, over a 10 year period.
Methods
The list of patients with acquired factor VIII inhibitor who were treated from June 2005 to May 2015 was identified and their case notes were then retrieved from the medical records of the hospital.
Results
Between 2005 and 2015, 28 patients from Kuala Lumpur were diagnosed with acquired factor VIII inhibitors. The population of Kuala Lumpur is 1,501,800 (Population Statistics 2003) giving an incidence of 1.8 cases /million/year.The patient demographics are shown in Table 1.Ten patients (36%) had an underlying associated disease. One was postpartum with history of psoriasis, two had systemic lupus erythematosus, three with bullous pemphigoid, two with thyroid disease, one rheumatoid arthritis and one with a malignant lung nodule. In the remaining 18 patients (64%) were idiopathic. All patients had abnormal bleeding with a total of 54 haemorrhagic episodes. 14 patients had life or limb threatening bleeding: postpartum haemorrhage, iliopsoas haematoma, compartment syndrome, arterial puncture, gastrointestinal bleed and intracranial bleed. Table 1. Demographics of patients with acquired FVIII inhibitor,
| Age(yrs) Mean (range) | 58.5(27-80) |
| Gender Male / Female | 16 /12 |
| Inhibitortitre(BU)Median(range) | 31.5(1.1-1404) |
| Median Inhibitor titre (BU) in life/limb threatening bleed | 9.7 |
| Median Inhibitor titre (BU) in subcutanoues bleed | 51.5 |
| Site of bleeding | No. of haemorrhages | Percentage (%) |
| Ecchymoses | 28 | 52 |
| Soft Tissue Haematomas | 5 | 9 |
| Haematuria | 3 | 5 |
| Intracrannial Bleed | 1 | 2 |
| Iliopsoas Hematoma | 3 | 5 |
| Other Intramuscular hematoma | 3 | 5 |
| Compartment Syndrome | 1 | 2 |
| Postpartum Haemorrhage | 1 | 2 |
| GI Bleeding | 5 | 9 |
| Bleeding post procedure / arterial puncture | 3 | 5 |
| Bleeding from injury | 1 | 2 |
Conclusion
Over a 10-year period we treated 28 patients with acquired hemophilia with an estimated incidence of 1.8/million/year. The median age of was 58.5 years, with a male preponderance(57%). It was associated with an underlying disease in 36% of cases. Life or limb threatening haemorrhages were seen in 50% of patients. Factor VIII level and inhibitor titre at presentation did not predict severity of bleeding.Mortality rate was 15%; all patients died of complications related to bleeding and treatment. It is important to recognize and diagnose this disorder, as appropriate treatment will give a favorable outcome; failing which it can be fatal.
Session topic: E-poster
Keyword(s): Acquired hemophilia
Abstract: E992
Type: Eposter Presentation
Background
Acquired Factor VIII inhibitor is a very rare bleeding condition due to the spontaneous development of autoantibodies directed against factor VIII.
Aims
To identify at the demographics, presenting features and bleeding characteristics of patients with Acquired Hemophilia referred to the Haematology Unit at Ampang General Hospital, over a 10 year period.
Methods
The list of patients with acquired factor VIII inhibitor who were treated from June 2005 to May 2015 was identified and their case notes were then retrieved from the medical records of the hospital.
Results
Between 2005 and 2015, 28 patients from Kuala Lumpur were diagnosed with acquired factor VIII inhibitors. The population of Kuala Lumpur is 1,501,800 (Population Statistics 2003) giving an incidence of 1.8 cases /million/year.The patient demographics are shown in Table 1.Ten patients (36%) had an underlying associated disease. One was postpartum with history of psoriasis, two had systemic lupus erythematosus, three with bullous pemphigoid, two with thyroid disease, one rheumatoid arthritis and one with a malignant lung nodule. In the remaining 18 patients (64%) were idiopathic. All patients had abnormal bleeding with a total of 54 haemorrhagic episodes. 14 patients had life or limb threatening bleeding: postpartum haemorrhage, iliopsoas haematoma, compartment syndrome, arterial puncture, gastrointestinal bleed and intracranial bleed. Table 1. Demographics of patients with acquired FVIII inhibitor,
Table 2. Sites of Haemorrhages on Presentation
Conclusion
Over a 10-year period we treated 28 patients with acquired hemophilia with an estimated incidence of 1.8/million/year. The median age of was 58.5 years, with a male preponderance(57%). It was associated with an underlying disease in 36% of cases. Life or limb threatening haemorrhages were seen in 50% of patients. Factor VIII level and inhibitor titre at presentation did not predict severity of bleeding.Mortality rate was 15%; all patients died of complications related to bleeding and treatment. It is important to recognize and diagnose this disorder, as appropriate treatment will give a favorable outcome; failing which it can be fatal.
Session topic: E-poster
Keyword(s): Acquired hemophilia
Type: Eposter Presentation
Background
Acquired Factor VIII inhibitor is a very rare bleeding condition due to the spontaneous development of autoantibodies directed against factor VIII.
Aims
To identify at the demographics, presenting features and bleeding characteristics of patients with Acquired Hemophilia referred to the Haematology Unit at Ampang General Hospital, over a 10 year period.
Methods
The list of patients with acquired factor VIII inhibitor who were treated from June 2005 to May 2015 was identified and their case notes were then retrieved from the medical records of the hospital.
Results
Between 2005 and 2015, 28 patients from Kuala Lumpur were diagnosed with acquired factor VIII inhibitors. The population of Kuala Lumpur is 1,501,800 (Population Statistics 2003) giving an incidence of 1.8 cases /million/year.The patient demographics are shown in Table 1.Ten patients (36%) had an underlying associated disease. One was postpartum with history of psoriasis, two had systemic lupus erythematosus, three with bullous pemphigoid, two with thyroid disease, one rheumatoid arthritis and one with a malignant lung nodule. In the remaining 18 patients (64%) were idiopathic. All patients had abnormal bleeding with a total of 54 haemorrhagic episodes. 14 patients had life or limb threatening bleeding: postpartum haemorrhage, iliopsoas haematoma, compartment syndrome, arterial puncture, gastrointestinal bleed and intracranial bleed. Table 1. Demographics of patients with acquired FVIII inhibitor,
| Age(yrs) Mean (range) | 58.5(27-80) |
| Gender Male / Female | 16 /12 |
| Inhibitortitre(BU)Median(range) | 31.5(1.1-1404) |
| Median Inhibitor titre (BU) in life/limb threatening bleed | 9.7 |
| Median Inhibitor titre (BU) in subcutanoues bleed | 51.5 |
| Site of bleeding | No. of haemorrhages | Percentage (%) |
| Ecchymoses | 28 | 52 |
| Soft Tissue Haematomas | 5 | 9 |
| Haematuria | 3 | 5 |
| Intracrannial Bleed | 1 | 2 |
| Iliopsoas Hematoma | 3 | 5 |
| Other Intramuscular hematoma | 3 | 5 |
| Compartment Syndrome | 1 | 2 |
| Postpartum Haemorrhage | 1 | 2 |
| GI Bleeding | 5 | 9 |
| Bleeding post procedure / arterial puncture | 3 | 5 |
| Bleeding from injury | 1 | 2 |
Conclusion
Over a 10-year period we treated 28 patients with acquired hemophilia with an estimated incidence of 1.8/million/year. The median age of was 58.5 years, with a male preponderance(57%). It was associated with an underlying disease in 36% of cases. Life or limb threatening haemorrhages were seen in 50% of patients. Factor VIII level and inhibitor titre at presentation did not predict severity of bleeding.Mortality rate was 15%; all patients died of complications related to bleeding and treatment. It is important to recognize and diagnose this disorder, as appropriate treatment will give a favorable outcome; failing which it can be fatal.
Session topic: E-poster
Keyword(s): Acquired hemophilia
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